2f:I[703058,["/_next/static/immutable/chunks/3bhq_5eh1d760.js","/_next/static/immutable/chunks/2iy5wruuedsh6.js","/_next/static/immutable/chunks/2pfsjpn6k6zlg.js","/_next/static/immutable/chunks/26c7v3_3uwnwu.js","/_next/static/immutable/chunks/00yc7rcvua6a7.js","/_next/static/immutable/chunks/37ktb71aq3480.js","/_next/static/immutable/chunks/2d50b-7eu4558.js","/_next/static/immutable/chunks/06_yt270h41rg.js","/_next/static/immutable/chunks/2d6noqpp6ehas.js","/_next/static/immutable/chunks/1sx186c93bpzg.js","/_next/static/immutable/chunks/3dc-1n866s65i.js","/_next/static/immutable/chunks/20x2y96ldu5wx.js","/_next/static/immutable/chunks/2w1bvggzb0ga8.js","/_next/static/immutable/chunks/2051ffx3xzio9.js"],"PaperListPageClient"] 30:T853,Multisystem Inflammatory Syndrome in Children (MIS-C) is a hyperinflammatory condition with multi-organ involvement, particularly affecting the cardiac and gastrointestinal systems. Although immunomodulatory therapy is standard, treatment approaches vary. This study aimed to evaluate treatment modalities in MIS-C such as methylprednisolone (MP), intravenous immunoglobulin (IVIG), anakinra and therapeutic plasma exchange (TPE) based on clinical severity and laboratory parameters in a prospectively followed cohort. A total of 125 MIS-C patients were included in the study and followed for at least 6 months after discharge. Patients were stratified by severity and treated with various immunomodulatory regimens, including IVIG+MP, IVIG+MP+anakinra, and IVIG+MP+anakinra+TPE. Patients with mild disease and low inflammatory markers (median CRP 9 mg/dL, ferritin 192 µg/dL) received IVIG+MP. Those with higher inflammation (CRP 20-24 mg/dL, ferritin 308-846 µg/dL) without cardio-pulmonary support were treated with IVIG+low-dose-MP+anakinra. Patients with shock, macrophage activation syndrome, or bicytopenia received IVIG+high-dose-MP+anakinra. TPE was added in cases requiring cardio-pulmonary support. Most were discharged without corticosteroids or anakinra; only 11% received a short outpatient prednisolone taper. The mid-term longitudinal assessment of MIS-C patients suggests that timely immunomodulatory therapies, guided by laboratory parameters, promote safe resolution of systemic inflammation and cardiac complications, and shorten treatment duration. Demonstrates that short-term, biomarker-guided use of anakinra and corticosteroids effectively controls hyperinflammation in MIS-C. Highlights that prolonged corticosteroid therapy may not be necessary, even in severe cases. Provides evidence of early cardiac recovery, including resolution of CAAs, without post-discharge steroids. Supports a steroid-sparing treatment approach, reducing risks of long-term immunosuppression. May inform future MIS-C treatment guidelines by minimizing the need for escalation therapy, ECMO, and related complications.31:Te4b,Abstract Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a hyperinflammatory condition with multi-organ involvement, particularly affecting the cardiac and gastrointestinal systems. Although immunomodulatory therapy is standard, treatment approaches vary. This study aimed to evaluate treatment modalities in MIS-C such as methylprednisolone (MP), intravenous immunoglobulin (IVIG), anakinra and therapeutic plasma exchange (TPE) based on clinical severity and laboratory parameters in a prospectively followed cohort. Methods A total of 125 MIS-C patients were included in the study and followed for at least 6 months after discharge. Patients were stratified by severity and treated with various immunomodulatory regimens, including IVIG+MP, IVIG+MP+anakinra, and IVIG+MP+anakinra+TPE. Results Patients with mild disease and low inflammatory markers (median CRP 9 mg/dL, ferritin 192 µg/dL) received IVIG+MP. Those with higher inflammation (CRP 20–24 mg/dL, ferritin 308–846 µg/dL) without cardio-pulmonary support were treated with IVIG+low-dose-MP+anakinra. Patients with shock, macrophage activation syndrome, or bicytopenia received IVIG+high-dose-MP+anakinra. TPE was added in cases requiring cardio-pulmonary support. Most were discharged without corticosteroids or anakinra; only 11% received a short outpatient prednisolone taper. Conclusion The mid-term longitudinal assessment of MIS-C patients suggests that timely immunomodulatory therapies, guided by laboratory parameters, promote safe resolution of systemic inflammation and cardiac complications, and shorten treatment duration. Impact

Demonstrates that short-term, biomarker-guided use of anakinra and corticosteroids effectively controls hyperinflammation in MIS-C.

Highlights that prolonged corticosteroid therapy may not be necessary, even in severe cases.

Provides evidence of early cardiac recovery, including resolution of CAAs, without post-discharge steroids.

Supports a steroid-sparing treatment approach, reducing risks of long-term immunosuppression.

May inform future MIS-C treatment guidelines by minimizing the need for escalation therapy, ECMO, and related complications.

32:T803,Kawasaki disease (KD) is the leading cause of acquired childhood heart disease, with untreated cases progressing to giant coronary artery aneurysms (GCAAs) in 2-3% of patients. While outcomes are well-documented in high-income settings, data from low- and middle-income countries (LMICs) remain limited. This study examines clinical profiles, treatment responses, and outcomes of children with GCAAs managed at a tertiary care center in South India. We conducted a retrospective review of 18 children with KD-associated GCAAs (z-score >10 or diameter >8mm) treated between 2019-2024 at a tertiary care center in Kerala, India. All patients were referred from external centers with documented IVIg resistance. Treatment intensification included infliximab, corticosteroids, cyclosporine, and anakinra. Primary outcome was GCAA dimension change at last follow-up; secondary outcomes included thrombotic events, mortality, and anticoagulation cessation. The cohort comprised predominantly of males (n = 14, 77.8%) with median symptom onset at 1.1 (IQR 0.3 - 2.7) years; 50% were under one year old. While ten children (55%) experienced delayed diagnosis (>10 days after symptom onset) with median diagnosis at 11 (IQR 7 - 13.5) days; all faced delays in treatment intensification. Excluding two children lost-to-follow-up, during the median follow-up of 8.6 (5.2 - 19.4) months, complete GCAA resolution occurred in three (18.8%), reduction to small/medium aneurysms in six (37.6%), while five (31.3%) remained static. One mortality (6.3%) from myocardial infarction and a patient requiring coronary artery bypass grafting were observed. Anticoagulation was discontinued in 56.25% of children whose aneurysms resolved or reduced significantly. Delayed diagnosis and treatment intensification may have contributed to mixed outcomes in this GCAA cohort. Early recognition, appropriate risk stratification, and timely immunosuppression intensification are essential to improve prognosis and reduce severe coronary complications in resource-limited settings.25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"methylprednisolone: generic Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on methylprednisolone (generic). Clinician-written summaries and actionable practice insights. 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FiO2 requirements stabilized beginning around DOL 148. The infant ultimately required tracheostomy (DOL 72) and lung transplantation, remaining mechanically ventilated throughout.","limitations":"- Single-patient case report; no ability to isolate CSA's contribution from concurrent hydroxychloroquine, glucocorticoids, and azithromycin.\n- The two specific ABCA3 variants have not been studied in the context of CSA correction in vitro, so mechanistic benefit is unconfirmed.\n- No discontinuation trial was attempted, so the impact of any individual agent on outcome is unknown.","practice_takeaway":"For infants with ABCA3 surfactant dysfunction unresponsive to standard therapy, CSA may be cautiously trialed starting at the lower end of transplant trough targets (100–150 ng/mL) to limit nephrotoxicity, given immature neonatal CYP3A4 (~10% of adult capacity at birth). Close renal monitoring and multidisciplinary input are essential before initiating CSA in neonates."}},{"doi":"10.1016/s2665-9913(26)00208-0","pmid":"42685740","title":"Derivation, validation, and proposed thresholds of the Lupus Arthritis and Musculoskeletal Disease Activity (LAMDA) instrument: a cohort study","journal_title":"The Lancet Rheumatology","journal_issn":"2665-9913","published_at":"2026-09-02T00:00:00.000Z","url":"https://doi.org/10.1016/s2665-9913(26)00208-0","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-08T06:32:00.754Z","updated_at":"2026-09-08T06:32:00.754Z","tags":[{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"condition","value":"Lupus Arthritis","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"condition","value":"Systemic Lupus Erythematosus","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"modality","value":"Glucocorticoid","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"sample_size","value":"n=177","value_numeric":177},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"warning","value":"Impact flag 'practice_changing' is provisional — the authors note that large multinational RCT validation is still underway as of publication.","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"warning","value":"Sample size tag (n=177) reflects the combined derivation (n=133) and external validation (n=44) cohorts; these are distinct, non-overlapping populations.","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00208-0","tag_type":"warning","value":"The specific intramuscular glucocorticoid used is not named in the abstract; methylprednisolone is assumed as it is standard practice but may warrant verification.","value_numeric":null}],"summaries":{"what_studied":"This cohort study derived and validated LAMDA, a novel composite lupus arthritis disease activity score, using penalised quantile regression against ultrasound synovitis (grey scale + power Doppler) in 133 SLE patients receiving intramuscular glucocorticoids (USEFUL study, 7 UK centres), then externally validated it in 44 patients at Leeds Teaching Hospitals.","key_result":"LAMDA retained 4 variables (swollen joint count in 66 joints, ESR, physician musculoskeletal VAS, and patient pain VAS) and was responsive to glucocorticoid therapy (effect size 0.40, p<0.0001). Proposed thresholds for trial entry, minimal disease activity, acceptable symptom state, and minimal clinically important improvement showed good logical consistency; thresholds outperformed swollen joint counts alone in discriminating treatment intention and acceptable symptom state in the external validation cohort (n=44).","limitations":"Both cohorts were predominantly female (~86–95%) and White (~62–73%), limiting generalisability. The derivation sample was relatively small (n=133), and external validation was modest (n=44). Further multinational RCT validation is still underway.","practice_takeaway":"LAMDA can be calculated using four readily available clinical variables — substituting swollen joint count in 28 joints for the 66-joint count is supported (ICC >0.99) — making it feasible for routine use. Clinicians running SLE arthritis trials should consider LAMDA over conventional joint counts alone, as it better captures ultrasound-verified synovitis and patient-relevant outcomes."}},{"doi":"10.1186/s12886-026-05199-5","pmid":"42638100","title":"Sympathetic ophthalmia complicating proliferative diabetic retinopathy: diagnostic and therapeutic challenges during pregnancy","journal_title":"BMC Ophthalmology","journal_issn":"1471-2415","published_at":"2026-08-24T00:00:00.000Z","url":"https://doi.org/10.1186/s12886-026-05199-5","source_score":7,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-02T06:22:41.110Z","updated_at":"2026-09-02T06:22:41.110Z","tags":[{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"brand","value":"Humira","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"category","value":"Ophthalmology","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"condition","value":"Proliferative Diabetic Retinopathy","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"condition","value":"Sympathetic Ophthalmia","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"condition","value":"Type 1 Diabetes Mellitus","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"condition","value":"Uveitis","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"generic","value":"adalimumab","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"generic","value":"bevacizumab","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"generic","value":"prednisolone","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"modality","value":"Anti-VEGF","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"modality","value":"Panretinal Photocoagulation","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"modality","value":"Pars Plana Vitrectomy","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"modality","value":"TNF Inhibitor","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"sample_size","value":"n=1","value_numeric":1},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"sex","value":"female","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"study_design","value":"Case Report","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"warning","value":"Adalimumab continuation into the third trimester was individualized given monocular status; this deviates from some guideline recommendations and should not be applied routinely.","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"warning","value":"Multiple confounding factors (SO, PDR, poor glycemic control, two pregnancies) coexist, making causal attribution of outcomes to any single intervention uncertain.","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"warning","value":"The Humira brand tag is inferred from adalimumab use; the paper does not specify the brand name explicitly.","value_numeric":null},{"paper_doi":"10.1186/s12886-026-05199-5","tag_type":"warning","value":"This is a single case report (n=1); findings should not be generalized to broader clinical practice without corroborating evidence.","value_numeric":null}],"summaries":{"what_studied":"A single case of a 27-year-old woman with poorly controlled type 1 diabetes who developed sympathetic ophthalmia (SO) in the fellow eye 10 weeks after penetrating ocular injury repair, with coexisting proliferative diabetic retinopathy (PDR) and a concurrent second pregnancy, managed with IV methylprednisolone, oral prednisolone taper, adalimumab, augmented panretinal photocoagulation (PRP), and postpartum pars plana vitrectomy.","key_result":"Visual acuity improved from 20/100 to 20/25 after systemic immunosuppression and augmented PRP, with partial regression of neovascularization; following postpartum vitrectomy for recurrent vitreous hemorrhage and tractional retinal detachment, final VA was 20/40 with inactive uveitis and a flat, attached retina.","limitations":"Single case report limits generalizability; multiple overlapping variables (SO, PDR, two pregnancies, poor glycemic control) make it impossible to isolate the contribution of each factor; no control or comparator eye with similar combined pathology.","practice_takeaway":"When SO is diagnosed in a patient with PDR, prompt systemic immunosuppression can itself reduce neovascular activity—consider early adalimumab as a steroid-sparing agent to limit corticosteroid-induced hyperglycemia. In pregnant patients, defer intravitreal anti-VEGF when possible, favor PRP, individualize biologic continuation with multidisciplinary input, and plan vitrectomy for the postpartum period unless vision-threatening urgency demands earlier intervention."}},{"doi":"10.1001/jamasurg.2026.3557","pmid":"42616512","title":"Preoperative High-Dose Corticosteroids in Digestive Cancer Surgery","journal_title":"JAMA Surgery","journal_issn":"2168-6254","published_at":"2026-08-19T00:00:00.000Z","url":"https://doi.org/10.1001/jamasurg.2026.3557","source_score":25,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-22T06:53:19.605Z","updated_at":"2026-08-22T06:53:19.605Z","tags":[{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"category","value":"Surgery","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"condition","value":"Digestive Cancer","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"condition","value":"Postoperative Complications","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"level_of_evidence","value":"1","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"sample_size","value":"n=1210","value_numeric":1210},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"study_design","value":"Randomized Controlled Trial","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"warning","value":"Hepatic-only surgery (without digestive anastomosis) was excluded; findings may not extend to liver resection for cancer.","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"warning","value":"The impact_flag 'practice_changing' is applied in the negative direction — this trial argues against a practice rather than establishing a new one.","value_numeric":null},{"paper_doi":"10.1001/jamasurg.2026.3557","tag_type":"warning","value":"The trial was conducted exclusively in French reference centers, which may have lower baseline complication rates than community hospitals.","value_numeric":null}],"summaries":{"what_studied":"This double-blind, placebo-controlled RCT (23 French centers, 2019–2023) assessed whether a single preoperative pulse of IV methylprednisolone (20 mg/kg at anesthesia induction) reduced major postoperative complications within 30 days in adults undergoing elective, curative-intent digestive cancer surgery.","key_result":"No significant benefit was found: major complications occurred in 23% (139/594) of the methylprednisolone group vs 20% (119/594) of placebo (P = .16). All secondary outcomes — postoperative infections (31% vs 30%), intra-abdominal infections (24% vs 21%), unsatisfactory wound healing (15% vs 12%), and mean hospital stay (~13.2 vs ~13.0 days) — were also non-significant.","limitations":"- Trial excluded patients undergoing hepatic surgery without digestive anastomosis, limiting generalizability to all digestive cancer operations.\n- Only French academic/reference centers participated, which may not reflect community practice outcomes.\n- A small number of patients (22 of 1210) were lost to follow-up, though this is unlikely to change conclusions.","practice_takeaway":"Do not routinely add a preoperative high-dose corticosteroid pulse to reduce complications in elective digestive cancer surgery — it provides no measurable benefit over placebo. This practice should be abandoned in this setting."}},{"doi":"10.1002/ppul.71762","pmid":"42528296","title":"Efficacy of High‐Dose Versus Conventional‐Dose Methylprednisolone in Reducing the Risk of Chronic Lung Disease in Chinese Children With Severe\n Mycoplasma pneumoniae\n Pneumonia: A Retrospective Study","journal_title":"Pediatric Pulmonology","journal_issn":"8755-6863","published_at":"2026-07-29T00:00:00.000Z","url":"https://pubmed.ncbi.nlm.nih.gov/42528296/","source_score":10,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:17:43.988Z","updated_at":"2026-08-04T18:17:43.988Z","tags":[{"paper_doi":"10.1002/ppul.71762","tag_type":"age_group","value":"pediatric","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"condition","value":"Chronic Lung Disease","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"condition","value":"Mycoplasma Pneumoniae Pneumonia","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"sample_size","value":"n=82","value_numeric":82},{"paper_doi":"10.1002/ppul.71762","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"warning","value":"Exact methylprednisolone doses defining 'high-dose' vs. 'conventional-dose' are not specified in the abstract; clinicians should consult the full paper for dosing details.","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"warning","value":"The 'impact_flag' of practice_changing is applied cautiously — the result is striking but comes from a retrospective, non-randomized, single-center study in a specific ethnic population; prospective validation is needed before changing standard of care.","value_numeric":null},{"paper_doi":"10.1002/ppul.71762","tag_type":"warning","value":"The very high CLD rate in the conventional-dose group (85.4%) is unusual and may reflect patient selection or local practice patterns specific to this center.","value_numeric":null}],"summaries":{"what_studied":"This retrospective cohort study compared high-dose vs. conventional-dose methylprednisolone in 82 Chinese children with severe *Mycoplasma pneumoniae* pneumonia (SMPP), admitted January 2018–January 2024, with outcomes assessed at 6-month follow-up.","key_result":"High-dose methylprednisolone led to shorter fever duration (9.8 vs. 16.1 days), faster defervescence after corticosteroid start (1.0 vs. 7.0 days), shorter hospitalization (13.4 vs. 18.4 days), and—most notably—a much lower rate of chronic lung disease at 6 months (28.2% vs. 85.4%; p < 0.001), along with better oxygen saturation and pulmonary function.","limitations":"Retrospective single-center design limits causal inference and generalizability. Dosing groups were not randomized, raising the risk of selection bias. The study was conducted exclusively in Chinese children, so findings may not extend to other populations.","practice_takeaway":"In children with SMPP, high-dose methylprednisolone appears to sharply cut the risk of chronic lung disease at 6 months compared to conventional dosing. Clinicians should weigh this potential long-term benefit against steroid risks while awaiting prospective trial data."}},{"doi":"10.1212/wnl.0000000000218300","pmid":"42485605","title":"Effectiveness and Safety of Plasmapheresis in MOGAD Attacks","journal_title":"Neurology","journal_issn":"0028-3878","published_at":"2026-07-22T00:00:00.000Z","url":"https://doi.org/10.1212/wnl.0000000000218300","source_score":16,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-28T14:02:35.600Z","updated_at":"2026-07-28T14:02:35.600Z","tags":[{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"category","value":"Clinical Neurology","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"condition","value":"Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"condition","value":"Myelitis","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"condition","value":"Optic Neuritis","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"modality","value":"Intravenous Immunoglobulin","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"modality","value":"Therapeutic Plasma Exchange","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"sample_size","value":"n=1045","value_numeric":1045},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"study_design","value":"Systematic Review and Meta-Analysis","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"warning","value":"Level of evidence set to 3 because pooled studies are observational (no RCTs); some may be case series, which could warrant a lower grade. Clinicians should interpret recovery rates cautiously given the very wide CIs (e.g., excellent recovery 33%–100%).","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"warning","value":"PROSPERO registration number appears to contain a formatting inconsistency (CRD420251169201) as transcribed from the abstract — verify against the registry if citing.","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000218300","tag_type":"warning","value":"The mean age (35 ± 17 years) spans a broad range potentially including adolescents; age group tagged as 'adult' based on the reported mean, but the study population may include pediatric patients.","value_numeric":null}],"summaries":{"what_studied":"This systematic review and meta-analysis evaluated the effectiveness and safety of plasmapheresis (versus IVMP or IVIG) for acute MOGAD attacks across 10 studies (1,045 patients, 1,368 attacks; mean age 35 ± 17 years, 56.5% female), with data from 8 studies pooled for analysis.","key_result":"In optic neuritis, plasmapheresis improved visual acuity (logMAR mean difference 1.43; 95% CI 0.63–2.24) with an excellent recovery rate of 81% (95% CI 33%–100%). For myelitis/cerebral involvement, good recovery reached 94% (95% CI 88%–100%). Adverse events were rare at 3% (95% CI 1%–14%).","limitations":"High heterogeneity across studies due to variable design, treatment timing, and attack severity. No randomized controlled trials were included. Wide confidence intervals (e.g., excellent recovery 33%–100%) limit precision of estimates.","practice_takeaway":"Plasmapheresis appears to be a safe escalation option for steroid-refractory MOGAD attacks, especially isolated optic neuritis. Clinicians should be aware that evidence remains observational and randomized trials are still needed before firm protocols can be established."}},{"doi":"10.1111/pai.70402","pmid":"42473072","title":"Oral food challenges in\n FPIES\n : A narrative review and proposals for emergency and home‐based management of acute\n FPIES\n reaction","journal_title":"Pediatric Allergy and Immunology","journal_issn":"0905-6157","published_at":"2026-07-19T00:00:00.000Z","url":"https://doi.org/10.1111/pai.70402","source_score":1,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-23T13:33:24.408Z","updated_at":"2026-07-23T13:33:24.408Z","tags":[{"paper_doi":"10.1111/pai.70402","tag_type":"age_group","value":"pediatric","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"brand","value":"Isofundin","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"brand","value":"Plasmalyte","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"brand","value":"Ringer Lactate","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"brand","value":"Zophren","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"category","value":"Allergy","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"condition","value":"Food Protein-Induced Enterocolitis Syndrome","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"generic","value":"ondansetron","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"modality","value":"Antiemetic","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"modality","value":"Intravenous Fluid Resuscitation","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"modality","value":"Oral Food Challenge","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"modality","value":"Oral Rehydration Therapy","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"sample_size","value":"n=31","value_numeric":31},{"paper_doi":"10.1111/pai.70402","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"warning","value":"Ondansetron dosing and indications described are partly off-label per French/FDA approvals (approved from 6 months for chemotherapy-related nausea; off-label use for FPIES is increasingly accepted but lacks RCT support.","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"warning","value":"The proposed OFC and emergency protocols are expert consensus from a French working group and have not yet been validated in a prospective trial; the French national registry (NCT05528900) is ongoing.","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"warning","value":"The sample_size tag reflects the number of studies included in the systematic search, not a patient-level N; individual study cohorts ranged from 4 to 267 OFCs.","value_numeric":null},{"paper_doi":"10.1111/pai.70402","tag_type":"warning","value":"The study design is a narrative review with a PRISMA-inspired (not full PRISMA) search methodology, which may introduce selection bias.","value_numeric":null}],"summaries":{"what_studied":"This narrative review (2017–2025, 31 studies) by the French FPIES working group evaluated oral food challenge (OFC) protocols and acute reaction management in children with Food Protein-Induced Enterocolitis Syndrome (FPIES), then proposed a standardized national protocol covering OFC dosing, hospital emergency care, and home emergency kits.","key_result":"A single dose of 25% of an age-appropriate serving size is sufficient to trigger a reaction in most cases (usually within 2–3 h). Split-dose one-day protocols carry significantly higher risk of severe reactions than multi-day protocols (e.g., one-day OFC: 30% severe vs. two-day OFC: 5% severe; OR=10.5, 95% CI 1.28–87.1). The proposed protocol uses 25% in hospital (day 1), then 50% and 100% at home on separate days. For acute reactions: oral/sublingual ondansetron for mild-to-moderate cases; IV isotonic fluid bolus 10 mL/kg over 5–20 min for moderate-to-severe; IV methylprednisolone 1 mg/kg for severe cases. Adrenaline is not indicated unless IgE-mediated features are present.","limitations":"Evidence base is predominantly retrospective studies with small sample sizes and heterogeneous protocols; no randomized controlled trials exist comparing OFC protocols or parenteral vs. oral/sublingual ondansetron. The proposed protocol is not yet validated prospectively (ongoing French registry NCT05528900).","practice_takeaway":"For supervised FPIES OFC, use a single 25% age-appropriate serving in hospital followed by home escalation (50%, then 100%)—this reduces severe reactions compared to multi-dose same-day protocols. Send families home with an emergency kit containing oral rehydration solution and weight-based oral ondansetron (2 mg for 8–15 kg, 4 mg for 15–30 kg, 8 mg for >30 kg); adrenaline is not needed in classic FPIES."}},{"doi":"10.1016/s2665-9913(26)00109-8","pmid":"42392130","title":"Long-term durability of a time-limited methotrexate intervention in patients with anti-citrullinated protein antibody-positive and anti-citrullinated protein antibody-negative arthralgia at increased risk for rheumatoid arthritis (TREAT EARLIER): 5-year data from a double-blind, randomised, placebo-controlled trial","journal_title":"The Lancet Rheumatology","journal_issn":"2665-9913","published_at":"2026-07-02T00:00:00.000Z","url":"https://doi.org/10.1016/s2665-9913(26)00109-8","source_score":5,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-12T13:19:41.791Z","updated_at":"2026-07-12T13:19:41.791Z","tags":[{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"condition","value":"Clinically Suspect Arthralgia","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"condition","value":"Rheumatoid Arthritis","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"generic","value":"methotrexate","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"modality","value":"Disease-Modifying Antirheumatic Drug","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"modality","value":"Glucocorticoid","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"sample_size","value":"n=236","value_numeric":236},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"study_design","value":"Randomized Controlled Trial","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"warning","value":"Level of evidence assigned as '2' (prospective RCT with long-term follow-up), though the key subgroup findings carry higher uncertainty due to post-hoc stratification.","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"warning","value":"The 5-year subgroup analysis by ACPA status and predicted risk (n=120) was a secondary/exploratory analysis; the original trial's primary endpoints were assessed at 2 years in the full cohort of 236 participants. Subgroup findings, particularly the strong ACPA-negative RA prevention result (NNT=4), should be interpreted with caution given small subgroup sizes and potential for type I error.","value_numeric":null},{"paper_doi":"10.1016/s2665-9913(26)00109-8","tag_type":"warning","value":"The ACPA-positive placebo subgroup was notably small (n=23), which may have underpowered the comparison in that stratum.","value_numeric":null}],"summaries":{"what_studied":"The TREAT EARLIER trial evaluated whether a 12-month methotrexate course (plus a single IM methylprednisolone 120 mg injection) versus placebo prevented rheumatoid arthritis (RA) and reduced physical disability over 5 years in adults with clinically suspect arthralgia and subclinical joint inflammation, stratified by ACPA status and predicted RA risk >10%.","key_result":"In ACPA-negative participants at increased risk, active treatment reduced RA development (9% vs 32%; HR 0.24 [95% CI 0.07–0.87], NNT=4) and sustained physical disability improvement (HAQ mean difference −0.16, p=0.0082) over 5 years. In ACPA-positive participants, neither RA prevention (58% vs 65%; HR 0.75, p=0.41) nor disability benefit (HAQ −0.12, p=0.12) was sustained.","limitations":"- The 5-year subgroup analysis (n=120 at increased predicted risk) was not pre-specified as the primary analysis, limiting causal inference.\n- Small subgroup sizes (especially ACPA-positive placebo group, n=23) reduce statistical power.\n- Single-country recruitment (13 Dutch centres) may limit generalisability.","practice_takeaway":"For ACPA-negative patients with clinically suspect arthralgia at >10% predicted RA risk, a time-limited course of methotrexate plus a glucocorticoid injection may meaningfully reduce both RA onset and disability over 5 years (NNT=4). This strategy does not appear to benefit ACPA-positive individuals, who likely need a different treatment approach."}},{"doi":"10.1186/s12890-026-04427-4","pmid":"42324522","title":"Mepolizumab initiation by day 30 and glucocorticoid exposure in newly diagnosed EGPA: a single-center 30-day landmark cohort study","journal_title":"BMC Pulmonary Medicine","journal_issn":"1471-2466","published_at":"2026-06-22T00:00:00.000Z","url":"https://doi.org/10.1186/s12890-026-04427-4","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-29T13:22:49.024Z","updated_at":"2026-06-29T13:22:49.024Z","tags":[{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"brand","value":"Nucala","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"condition","value":"Eosinophilic Granulomatosis With Polyangiitis","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"generic","value":"mepolizumab","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"generic","value":"prednisolone","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"modality","value":"Anti-IL-5 Monoclonal Antibody","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"modality","value":"Glucocorticoid","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"sample_size","value":"n=27","value_numeric":27},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"warning","value":"Calendar-time confounding is a particular concern as mepolizumab became more widely available over time, which the authors acknowledge but cannot fully adjust for.","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"warning","value":"Sex distribution is not explicitly reported in the abstract; 'mixed' is assumed based on typical EGPA cohort composition.","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"warning","value":"The landmark cohort includes only 6 patients in the early mepolizumab group, making all effect estimates highly imprecise and potentially unreliable.","value_numeric":null},{"paper_doi":"10.1186/s12890-026-04427-4","tag_type":"warning","value":"The study is described as a pre-publication unedited manuscript; final content may differ after editorial revision.","value_numeric":null}],"summaries":{"what_studied":"This single-center pilot retrospective cohort study examined whether starting mepolizumab within 30 days of glucocorticoid initiation (vs. later or never) reduces oral prednisolone exposure in 27 newly diagnosed EGPA patients, followed to 12 months using a landmark cohort design.","key_result":"Cumulative oral prednisolone exposure (day 30–12 months) was markedly lower in the early mepolizumab group vs. the delayed/no-mepolizumab group (24.7 vs. 68.6 mg/kg); similar reductions were seen at 6 months (17.6 vs. 43.6 mg/kg). Disease activity (BVAS = 0) was achieved by 3 months in all evaluable patients in both groups.","limitations":"- Very small groups (n=6 early mepolizumab vs. n=21 comparator) severely limit statistical power and precision.\n- Nonrandomized design with unmeasured confounding from calendar time, treatment indication, and concomitant therapies.\n- Between-group differences were attenuated when IV methylprednisolone pulse exposure was included, suggesting oral prednisolone alone may not fully capture total steroid burden.","practice_takeaway":"Early mepolizumab (within 30 days) was associated with roughly 60% less cumulative oral prednisolone at 12 months, but this is hypothesis-generating only—confounding cannot be ruled out in this small pilot study. Larger prospective studies are needed before changing practice."}},{"doi":"10.1212/nxi.0000000000200597","pmid":"42214057","title":"Clinical Manifestations, Tumor Associations, and Long-Term Outcomes of Anti-KLHL11 Encephalitis","journal_title":"Neurology Neuroimmunology & Neuroinflammation","journal_issn":"2332-7812","published_at":"2026-05-29T00:00:00.000Z","url":"https://doi.org/10.1212/nxi.0000000000200597","source_score":13,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-10T14:24:36.007Z","updated_at":"2026-06-10T14:24:36.007Z","tags":[{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"category","value":"Neurosciences","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Anti-KLHL11 Encephalitis","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Brainstem Encephalitis","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Cerebellar Ataxia","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Limbic Encephalitis","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Opsoclonus-Myoclonus Syndrome","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"condition","value":"Paraneoplastic Neurologic Syndrome","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"generic","value":"cyclophosphamide","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"generic","value":"rituximab","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"modality","value":"Corticosteroid","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"modality","value":"Immunotherapy","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"modality","value":"Intravenous Immunoglobulin","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"modality","value":"Plasma Exchange","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"modality","value":"Rituximab","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"sample_size","value":"n=17","value_numeric":17},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"study_design","value":"Retrospective and Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"warning","value":"Sample size is only 17 patients; all effect estimates and percentages should be interpreted with caution due to very wide uncertainty intervals.","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"warning","value":"Study was conducted at a single tertiary referral center (Erasmus MC), limiting generalizability.","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"warning","value":"The cohort was enriched for classic PNS features and antibodies during retrospective screening, introducing referral bias — true population incidence and phenotype distribution may differ.","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"warning","value":"The novel tumor associations (renal cell carcinoma, urothelial carcinoma) are each represented by only 1 patient; causal relationship with KLHL11 autoimmunity is unconfirmed.","value_numeric":null},{"paper_doi":"10.1212/nxi.0000000000200597","tag_type":"warning","value":"Two patients in the cohort had no available serum, only CSF, so antibody detection may be underestimated in serum-only testing workflows.","value_numeric":null}],"summaries":{"what_studied":"A Dutch cohort study (retrospective + prospective, Jan 2010–Jul 2023) screened 1,361 patients to identify and characterize all cases of anti-KLHL11 encephalitis, describing clinical phenotypes, tumor associations, treatment responses, and long-term outcomes in 17 confirmed patients.","key_result":"17 patients identified (71% male, median age 59); leading phenotypes were cerebellar ataxia and brainstem encephalitis (each 71%), opsoclonus-myoclonus syndrome (47%), and limbic encephalitis (18%). Tumors found in 59% (seminoma most common, 29%). After immunotherapy, 67% improved or stabilized (47% improved, 20% stabilized). Six patients (35%) died within 12 months — 3 from encephalitis, 3 from cancer. Median mRS at last follow-up was 4.","limitations":"- Very small sample (n=17) limits statistical power and generalizability.\n- Retrospective design with referral bias toward more severe cases and incomplete data.\n- No treatment consensus or standardized protocol, introducing confounding in efficacy assessment.","practice_takeaway":"Screen for anti-KLHL11 encephalitis in any patient with new brainstem/cerebellar syndrome, opsoclonus-myoclonus, or acute vestibulopathy — not just young men with seminoma, as urological tumors and atypical phenotypes (limbic encephalitis, meningitis-like) also occur. Testicular ultrasound outperforms CT and FDG-PET for detecting seminoma, and early combined immunotherapy plus tumor treatment offers the best chance of stabilization."}},{"doi":"10.3389/fped.2026.1812770","pmid":null,"title":"Case Report: Ruxolitinib for systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome: two pediatric cases and literature review","journal_title":"Frontiers in Pediatrics","journal_issn":"2296-2360","published_at":"2026-05-28T00:00:00.000Z","url":"https://doi.org/10.3389/fped.2026.1812770","source_score":4,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":false,"crossref":true,"unpaywall":true},"created_at":"2026-05-30T13:22:55.879Z","updated_at":"2026-05-30T13:22:55.879Z","tags":[{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"age_group","value":"pediatric","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"category","value":"Pediatrics","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"condition","value":"Macrophage Activation Syndrome","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"condition","value":"Systemic Juvenile Idiopathic Arthritis","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"generic","value":"canakinumab","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"generic","value":"ruxolitinib","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"generic","value":"tocilizumab","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"modality","value":"JAK Inhibitor","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"sample_size","value":"n=7","value_numeric":7},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"study_design","value":"Case Report with Literature Review","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"warning","value":"Concomitant use of plasma exchange in some patients may have reduced ruxolitinib drug levels, making efficacy attribution uncertain.","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"warning","value":"One serious adverse event (EBV-HLH with agranulocytosis) occurred, suggesting a meaningful safety signal in this immunocompromised population despite the small sample.","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"warning","value":"Study design is listed as 'Case Report' but functions as a retrospective case series plus narrative review — level of evidence assigned accordingly.","value_numeric":null},{"paper_doi":"10.3389/fped.2026.1812770","tag_type":"warning","value":"Total cohort of 7 patients includes only 2 new cases; the remaining 5 are from prior publications, so the literature review component carries cumulative selection and publication bias.","value_numeric":null}],"summaries":{"what_studied":"Efficacy and safety of ruxolitinib (JAK1/2 inhibitor) in pediatric sJIA-MAS, combining two new cases (ages 19 months and 4 years) with a literature review of 5 previously reported cases — totaling 7 children aged 1–11 years, most with refractory disease failing high-dose glucocorticoids and/or biologics.","key_result":"5 of 7 patients achieved complete remission and 2 achieved partial remission; no deaths occurred. Both new cases normalized temperature within 2–3 days of ruxolitinib and discontinued glucocorticoids within 3–6 months. One patient developed EBV-associated HLH and agranulocytosis, resolving after drug discontinuation; transient neutropenia occurred in 2 others.","limitations":"- Very small sample (n=7 case reports/series); publication bias likely.\n- Most patients received concomitant therapies (tocilizumab, cyclosporine, plasma exchange), making it impossible to isolate ruxolitinib's independent effect.\n- Plasma exchange may alter ruxolitinib pharmacokinetics, further complicating efficacy assessment.","practice_takeaway":"Consider ruxolitinib as rescue or bridging therapy for refractory sJIA-MAS when standard glucocorticoids and IL-1/IL-6 blockers fail — but monitor closely for cytopenia and viral reactivation (especially EBV/CMV). After acute control, IL-1 or IL-6–targeted therapy should remain the backbone for long-term sJIA management."}},{"doi":"10.1212/wnl.0000000000214933","pmid":"42139653","title":"Autoimmune Encephalitis as Treatment-Responsive Cause of Rapidly Progressive Dementia","journal_title":"Neurology","journal_issn":"0028-3878","published_at":"2026-05-15T00:00:00.000Z","url":"https://doi.org/10.1212/wnl.0000000000214933","source_score":12,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-25T13:25:27.613Z","updated_at":"2026-06-25T13:25:27.613Z","tags":[{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"category","value":"Clinical Neurology","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"condition","value":"Anti-LGI1 Encephalitis","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"condition","value":"Autoimmune Encephalitis","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"condition","value":"Autoimmune GFAP Astrocytopathy","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"condition","value":"Creutzfeldt-Jakob Disease","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"condition","value":"Rapidly Progressive Dementia","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"generic","value":"intravenous immunoglobulin","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"generic","value":"rituximab","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"modality","value":"Immunotherapy","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"sample_size","value":"n=147","value_numeric":147},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"warning","value":"Autopsy confirmation was available in only 8 of 147 patients (5%), so some diagnostic classifications rest on clinical consensus rather than neuropathology.","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"warning","value":"Seronegative AE (SN-AE) is a heterogeneous category without specific biomarkers; its inclusion relies on 2016 clinical criteria applied rigorously, which may not be reproducible across all centers.","value_numeric":null},{"paper_doi":"10.1212/wnl.0000000000214933","tag_type":"warning","value":"The study was conducted at the Dutch national AE referral center; the 39% AE frequency is likely higher than in general or prion-focused settings and should not be directly extrapolated.","value_numeric":null}],"summaries":{"what_studied":"This prospective multicenter cohort study (Netherlands, Dec 2019–Dec 2024) enrolled 147 adults with rapidly progressive dementia (RPD, defined as dementia onset within 1 year of symptoms) to determine the frequency and clinical subphenotypes of autoimmune encephalitis (AE) as a cause of RPD, compared to neurodegenerative and prion diseases.","key_result":"AE was the single largest diagnostic category (58/147; 39%) and the most common treatment-responsive cause of RPD (58/95; 61%). Anti-LGI1 (33% of AE-RPD), seronegative AE (19%), and autoimmune GFAP astrocytopathy (14%) were the top subtypes. Seizures at first presentation strongly favored AE over other diagnoses (34% vs 10%; p<0.001), with anti-LGI1 driving 55% of those seizure cases. Movement disorders emerging within 3 months of onset also pointed toward AE over CJD (80% vs 25%; p=0.004).","limitations":"- Single-country referral center bias (EMC is the Dutch national AE center), likely inflating AE frequency vs other settings such as prion or infection-focused centers.\n- Typical CJD cases may have been under-referred, reducing RT-QuIC sensitivity and skewing the diagnostic mix.\n- Neuropsychological testing was not systematically performed due to severity of deficits, limiting cognitive profiling across diagnoses.","practice_takeaway":"In any patient with RPD, test serum and CSF for a full neuronal/glial autoantibody panel—including GFAP in CSF—because AE is the most common treatable cause and early immunotherapy improves outcomes. Be alert for subtle focal seizures (present in 42% of AE-RPD seizure cases) and for movement disorders appearing within the first 3 months, both of which strongly favor AE over CJD."}},{"doi":"10.1038/s41390-025-04706-6","pmid":"41519951","title":"Multisystem Inflammatory Syndrome in Children with tailored therapy and six-month outcome","journal_title":"Pediatric Research","journal_issn":"0031-3998","published_at":"2026-01-11T00:00:00.000Z","url":"https://doi.org/10.1038/s41390-025-04706-6","source_score":21,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-01-12","doi":"10.1038/s41390-025-04706-6","days":"7","issn":"0031-3998","rank":"6","field":"Clinical Medicine","score":"21","category":"Pediatrics","categories":["Pediatrics"],"_categories":["Pediatrics"]},"pubmed":{"doi":"10.1038/s41390-025-04706-6","title":"Multisystem Inflammatory Syndrome in Children with tailored therapy and six-month outcome.","journal":"Pediatric research","methods":"","results":"Patients with mild disease and low inflammatory markers (median CRP 9 mg/dL, ferritin 192 µg/dL) received IVIG+MP. Those with higher inflammation (CRP 20-24 mg/dL, ferritin 308-846 µg/dL) without cardio-pulmonary support were treated with IVIG+low-dose-MP+anakinra. Patients with shock, macrophage activation syndrome, or bicytopenia received IVIG+high-dose-MP+anakinra. TPE was added in cases requiring cardio-pulmonary support. Most were discharged without corticosteroids or anakinra; only 11% received a short outpatient prednisolone taper.","abstract":"$30","keywords":[],"pubmed_id":"41519951","conclusions":"The mid-term longitudinal assessment of MIS-C patients suggests that timely immunomodulatory therapies, guided by laboratory parameters, promote safe resolution of systemic inflammation and cardiac complications, and shorten treatment duration.","publication_date":"2026-01-11T00:00:00"},"crossref":{"DOI":"10.1038/s41390-025-04706-6","URL":"https://doi.org/10.1038/s41390-025-04706-6","ISSN":["0031-3998","1530-0447"],"link":[{"URL":"https://www.nature.com/articles/s41390-025-04706-6.pdf","content-type":"application/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https://www.nature.com/articles/s41390-025-04706-6","content-type":"text/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https://www.nature.com/articles/s41390-025-04706-6.pdf","content-type":"application/pdf","content-version":"vor","intended-application":"similarity-checking"}],"page":null,"type":"journal-article","issue":null,"score":1,"title":["Multisystem Inflammatory Syndrome in Children with tailored therapy and six-month outcome"],"author":[{"given":"Osman Oguz","family":"Demir","sequence":"first","affiliation":[]},{"given":"Kubra","family":"Aykac","sequence":"additional","affiliation":[]},{"given":"Arthur Hoi Hin","family":"Cheng","sequence":"additional","affiliation":[]},{"given":"Selman","family":"Kesici","sequence":"additional","affiliation":[]},{"given":"H. Hakan","family":"Aykan","sequence":"additional","affiliation":[]},{"given":"Yelda","family":"Bilginer","sequence":"additional","affiliation":[]},{"given":"Ali Bulent","family":"Cengiz","sequence":"additional","affiliation":[]},{"given":"Rae S. M.","family":"Yeung","sequence":"additional","affiliation":[]},{"given":"Yasemin","family":"Ozsurekci","sequence":"additional","affiliation":[]},{"given":"Seza","family":"Ozen","sequence":"additional","affiliation":[]}],"issued":{"date-parts":[[2026,1,10]]},"member":"297","prefix":"10.1038","source":"Crossref","volume":null,"created":{"date-time":"2026-01-10T20:15:19Z","timestamp":1768076119000,"date-parts":[[2026,1,10]]},"indexed":{"date-time":"2026-01-11T23:51:19Z","timestamp":1768175479004,"date-parts":[[2026,1,11]]},"license":[{"URL":"https://creativecommons.org/licenses/by/4.0","start":{"date-time":"2026-01-10T00:00:00Z","timestamp":1768003200000,"date-parts":[[2026,1,10]]},"delay-in-days":0,"content-version":"tdm"},{"URL":"https://creativecommons.org/licenses/by/4.0","start":{"date-time":"2026-01-10T00:00:00Z","timestamp":1768003200000,"date-parts":[[2026,1,10]]},"delay-in-days":0,"content-version":"vor"}],"subject":[],"abstract":"$31","language":"en","relation":{},"resource":{"primary":{"URL":"https://www.nature.com/articles/s41390-025-04706-6"}},"subtitle":[],"assertion":[{"name":"received","group":{"name":"ArticleHistory","label":"Article History"},"label":"Received","order":1,"value":"13 May 2025"},{"name":"revised","group":{"name":"ArticleHistory","label":"Article History"},"label":"Revised","order":2,"value":"28 August 2025"},{"name":"accepted","group":{"name":"ArticleHistory","label":"Article History"},"label":"Accepted","order":3,"value":"20 November 2025"},{"name":"first_online","group":{"name":"ArticleHistory","label":"Article History"},"label":"First Online","order":4,"value":"10 January 2026"},{"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"},"order":1,"value":"The authors declare no competing interests."}],"deposited":{"date-time":"2026-01-11T21:07:19Z","timestamp":1768165639000,"date-parts":[[2026,1,11]]},"issn-type":[{"type":"print","value":"0031-3998"},{"type":"electronic","value":"1530-0447"}],"published":{"date-parts":[[2026,1,10]]},"publisher":"Springer Science and Business Media LLC","reference":[{"DOI":"10.1016/S0140-6736(20)31094-1","key":"4706_CR1","year":"2020","issue":null,"author":"S Riphagen","volume":"395","first-page":"1607","unstructured":"Riphagen, S., Gomez, X., Gonzalez-Martinez, C., Wilkinson, N. & Theocharis, P. Hyperinflammatory shock in children during COVID-19 pandemic. Lancet 395, 1607–1608 (2020).","article-title":null,"journal-title":"Lancet","doi-asserted-by":"publisher"},{"DOI":"10.1001/jama.2020.10369","key":"4706_CR2","year":"2020","issue":null,"author":"E Whittaker","volume":"324","first-page":"259","unstructured":"Whittaker, E. et al. Clinical characteristics of 58 children with a pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2. JAMA 324, 259–269 (2020).","article-title":null,"journal-title":"JAMA","doi-asserted-by":"publisher"},{"DOI":null,"key":"4706_CR3","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Centers for Disease Control and Prevention. 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Treatment intensity was guided by CRP and ferritin (mild: CRP 9 mg/dL, ferritin 192 µg/dL; higher inflammation without cardio-pulmonary support: CRP 20–24 mg/dL, ferritin 308–846 µg/dL; shock/MAS/bicytopenia or cardio-pulmonary support prompted escalation including high-dose MP, anakinra, and TPE).","limitations":"Severity-guided treatment allocation in this prospective cohort limits causal inference between regimens. The abstract provides few outcome metrics (e.g., no event rates for cardiac recovery, ICU/ECMO use, or adverse events).","practice_takeaway":"When treating MIS-C, use CRP, ferritin, and clinical severity to tailor short-course IVIG+MP±anakinra, reserving TPE for cardio-pulmonary support. Prolonged post-discharge steroids may be unnecessary, as only 11% needed a brief taper."}},{"doi":"10.1186/s12969-025-01178-0","pmid":"41462272","title":"Clinical outcomes of giant coronary aneurysms in South Asian children with Kawasaki disease","journal_title":"Pediatric Rheumatology","journal_issn":"1546-0096","published_at":"2025-12-30T00:00:00.000Z","url":"https://doi.org/10.1186/s12969-025-01178-0","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-01-04","doi":"10.1186/s12969-025-01178-0","days":"14","issn":"1546-0096","rank":"10","field":"Clinical Medicine","score":"2","category":"Rheumatology","categories":["Rheumatology"],"_categories":["Rheumatology"]},"pubmed":{"doi":"10.1186/s12969-025-01178-0","title":"Clinical outcomes of giant coronary aneurysms in South Asian children with Kawasaki disease.","journal":"Pediatric rheumatology online journal","methods":"","results":"The cohort comprised predominantly of males (n = 14, 77.8%) with median symptom onset at 1.1 (IQR 0.3 - 2.7) years; 50% were under one year old. While ten children (55%) experienced delayed diagnosis (>10 days after symptom onset) with median diagnosis at 11 (IQR 7 - 13.5) days; all faced delays in treatment intensification. Excluding two children lost-to-follow-up, during the median follow-up of 8.6 (5.2 - 19.4) months, complete GCAA resolution occurred in three (18.8%), reduction to small/medium aneurysms in six (37.6%), while five (31.3%) remained static. One mortality (6.3%) from myocardial infarction and a patient requiring coronary artery bypass grafting were observed. Anticoagulation was discontinued in 56.25% of children whose aneurysms resolved or reduced significantly.","abstract":"$32","keywords":["Child","Coronary aneurysm","Intravenous immunoglobulins","Mucocutaneous lymph node syndrome","Retrospective studies","Treatment outcome","Vasculitis"],"pubmed_id":"41462272","conclusions":"Delayed diagnosis and treatment intensification may have contributed to mixed outcomes in this GCAA cohort. Early recognition, appropriate risk stratification, and timely immunosuppression intensification are essential to improve prognosis and reduce severe coronary complications in resource-limited settings.","publication_date":"2025-12-30T00:00:00"},"crossref":{"DOI":"10.1186/s12969-025-01178-0","URL":"https://doi.org/10.1186/s12969-025-01178-0","ISSN":["1546-0096"],"link":[{"URL":"https://link.springer.com/content/pdf/10.1186/s12969-025-01178-0.pdf","content-type":"application/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https://link.springer.com/article/10.1186/s12969-025-01178-0","content-type":"text/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https://link.springer.com/content/pdf/10.1186/s12969-025-01178-0.pdf","content-type":"application/pdf","content-version":"vor","intended-application":"similarity-checking"}],"page":null,"type":"journal-article","issue":"1","score":1,"title":["Clinical outcomes of giant coronary aneurysms in South Asian children with Kawasaki disease"],"author":[{"given":"Kumar","family":"Abhinav","sequence":"first","affiliation":[]},{"given":"Nisanth","family":"Selvam","sequence":"additional","affiliation":[]},{"given":"Raman Krishna","family":"Kumar","sequence":"additional","affiliation":[]},{"given":"Aishee","family":"Bhattacharya","sequence":"additional","affiliation":[]},{"given":"Manu","family":"Pradeep","sequence":"additional","affiliation":[]},{"given":"Suma","family":"Balan","sequence":"additional","affiliation":[]}],"issued":{"date-parts":[[2025,12,29]]},"member":"297","prefix":"10.1186","source":"Crossref","volume":"23","created":{"date-time":"2025-12-29T17:16:53Z","timestamp":1767028613000,"date-parts":[[2025,12,29]]},"indexed":{"date-time":"2025-12-29T17:18:57Z","timestamp":1767028737819,"date-parts":[[2025,12,29]]},"license":[{"URL":"https://creativecommons.org/licenses/by-nc-nd/4.0","start":{"date-time":"2025-12-29T00:00:00Z","timestamp":1766966400000,"date-parts":[[2025,12,29]]},"delay-in-days":0,"content-version":"tdm"},{"URL":"https://creativecommons.org/licenses/by-nc-nd/4.0","start":{"date-time":"2025-12-29T00:00:00Z","timestamp":1766966400000,"date-parts":[[2025,12,29]]},"delay-in-days":0,"content-version":"vor"}],"subject":[],"abstract":null,"language":"en","relation":{},"resource":{"primary":{"URL":"https://link.springer.com/10.1186/s12969-025-01178-0"}},"subtitle":[],"assertion":[{"name":"received","group":{"name":"ArticleHistory","label":"Article History"},"label":"Received","order":1,"value":"20 July 2025"},{"name":"accepted","group":{"name":"ArticleHistory","label":"Article History"},"label":"Accepted","order":2,"value":"16 November 2025"},{"name":"first_online","group":{"name":"ArticleHistory","label":"Article History"},"label":"First Online","order":3,"value":"29 December 2025"},{"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"},"order":1},{"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"},"order":2,"value":"This study was performed in line with the principles of the Declaration of Helsinki. 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Primary outcome was change in aneurysm size at last follow-up; median follow-up among 16 with data was 8.6 months (IQR 5.2–19.4).","key_result":"Among 16 with follow-up, complete resolution occurred in 3/16 (18.8%); reduction to small or medium aneurysms in 6/16 (37.5%); and 5/16 (31.3%) remained giant. There were 2 myocardial infarctions, 1 death (6.3%), 1 CABG, and anticoagulation was stopped in 9/16 (56.25%) whose aneurysms regressed.","limitations":"Single-center retrospective series with small sample (n=18) and referral bias toward severe cases; short follow-up with two lost to follow-up; institutional risk stratification and expanded IVIg-resistance criteria were unvalidated.","practice_takeaway":"In this LMIC cohort, mixed outcomes and a death occurred, with many children diagnosed after day 10 and intensification delayed, underscoring the need for early recognition, risk stratification, serial echocardiography, and timely step-up immunosuppression in high-risk or IVIg-resistant Kawasaki disease."}},{"doi":"10.2340/actadv.v105.44373","pmid":"41024729","title":"Cutaneous Graft-Versus-Host Disease Lichen Spinulosus-like: a New Form of Presentation of an Old Entity","journal_title":"Acta Dermato-Venereologica","journal_issn":"1651-2057","published_at":"2025-09-30T00:00:00.000Z","url":"https://doi.org/10.2340/actadv.v105.44373","source_score":1,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2025-10-02","doi":"10.2340/actadv.v105.44373","days":"7","issn":"0001-5555","rank":"8","field":"Clinical Medicine","score":"1","category":"Dermatology","categories":["Dermatology"],"_categories":["Dermatology"]},"pubmed":{"doi":"10.2340/actadv.v105.44373","title":"Cutaneous Graft-Versus-Host Disease Lichen Spinulosus-like: a New Form of Presentation of an Old Entity.","journal":"Acta dermato-venereologica","methods":"","results":"","abstract":"","keywords":[],"pubmed_id":"41024729","conclusions":"","publication_date":"2025-09-30T00:00:00"},"crossref":{"DOI":"10.2340/actadv.v105.44373","URL":"https://doi.org/10.2340/actadv.v105.44373","ISSN":["1651-2057","0001-5555"],"link":[{"URL":"https://medicaljournalssweden.se/actadv/article/download/44373/51593","content-type":"application/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https://medicaljournalssweden.se/actadv/article/download/44373/51593","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":"adv44373","type":"journal-article","issue":null,"score":1,"title":["Cutaneous Graft-Versus-Host Disease Lichen Spinulosus-like: a New Form of Presentation of an Old Entity"],"author":[{"given":"Andrés","family":"Vidal González","sequence":"first","affiliation":[]},{"given":"Laura","family":"Taboada Paz","sequence":"additional","affiliation":[]},{"given":"Sergio","family":"López Alcázar","sequence":"additional","affiliation":[]},{"given":"Luis","family":"Requena Caballero","sequence":"additional","affiliation":[]},{"given":"Marta","family":"Feito Rodríguez","sequence":"additional","affiliation":[]}],"issued":{"date-parts":[[2025,9,30]]},"member":"1183","prefix":"10.2340","source":"Crossref","volume":"105","created":{"date-time":"2025-09-30T07:54:48Z","timestamp":1759218888000,"date-parts":[[2025,9,30]]},"indexed":{"date-time":"2025-10-01T15:53:14Z","timestamp":1759333994914,"date-parts":[[2025,10,1]]},"license":[{"URL":"https://creativecommons.org/licenses/by-nc/4.0","start":{"date-time":"2025-09-30T00:00:00Z","timestamp":1759190400000,"date-parts":[[2025,9,30]]},"delay-in-days":0,"content-version":"unspecified"}],"subject":[],"abstract":null,"relation":{},"resource":{"primary":{"URL":"https://medicaljournalssweden.se/actadv/article/view/44373"}},"subtitle":[],"deposited":{"date-time":"2025-09-30T07:54:53Z","timestamp":1759218893000,"date-parts":[[2025,9,30]]},"issn-type":[{"type":"electronic","value":"1651-2057"},{"type":"print","value":"0001-5555"}],"published":{"date-parts":[[2025,9,30]]},"publisher":"MJS Publishing, Medical Journals Sweden AB","reference":[{"DOI":"10.1111/j.1365-4632.2010.04552.x","key":"128115","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Kim S, Choi J, Kim J, Cho B, Kim DW, Park H. Clinicopathologic characteristics of cutaneous chronic graft-versus- host diseases: a retrospective study in Korean patients. Int J Dermatology 2010; 49: 1386–1392.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"},{"DOI":"10.1016/j.blre.2023.101054","key":"128116","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Haroun E, Agrawal K, Leibovitch J, Kassab J, Zoghbi M, Dutta D, Lim SH. Chronic graft- versus-host disease in pediatric patients: differences and challenges. Blood Rev 2023; 60: 101054.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"},{"DOI":"10.1016/j.jtct.2021.06.001","key":"128117","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Wolff D, Radojcic V, Lafyatis R, Cinar R, Rosenstein RK, Cowen EW et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: IV. The 2020 Highly morbid forms report. Transplant Cell Ther 2021; 27: 817–835.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"},{"DOI":"10.1016/0190-9622(95)91542-7","key":"128118","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Requena L, Sarasa JL, Masllorens FO, et al. Follicular spicules of the nose: a peculiar cutaneous manifestation of multiple myeloma with cryoglobulinemia. J Am Acad Dermatol 1995; 32: 834–839.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"},{"DOI":"10.1016/j.jaad.2014.12.022","key":"128119","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Cornejo CM, Kim EJ, Rosenbach M, Micheletti RG. Atypical manifestations of graft-versus-host disease. 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Parafollicular hair bulge in human GVHD: a stem cell-rich primary target. Bone Marrow Transplant 1993; 11: 223–225.","article-title":null,"journal-title":null,"doi-asserted-by":null},{"DOI":"10.1111/cup.12391","key":"128123","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Llamas-Velasco M, Solano-López Morel GE, Gruber-Wackernagel A, Concha-Garzón MJ, Requena L, Cerroni L. Comedonal graft-vs-host disease: a distinct clinical expression of a lichenoid follicular GVHD. J Cutan Pathol 2014; 41: 853–858.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"},{"DOI":"10.4103/idoj.IDOJ_87_20","key":"128124","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Vashisht D, Kothari R, Baveja S, Neema S, Sengupta P, Sandhu S. Follicular graft vs host reaction: a rare presentation. 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Am J Clin Dermatol 2018; 19: 33–50.","article-title":null,"journal-title":null,"doi-asserted-by":"crossref"}],"short-title":[],"content-domain":{"domain":[],"crossmark-restriction":false},"original-title":[],"container-title":["Acta Dermato-Venereologica"],"reference-count":15,"published-online":{"date-time":null,"timestamp":null,"date-parts":[[2025,9,30]]},"references-count":15,"short-container-title":["Acta Derm Venereol"],"is-referenced-by-count":0},"unpaywall":{"doi":"10.2340/actadv.v105.44373","is_oa":true,"title":"Cutaneous Graft-Versus-Host Disease Lichen Spinulosus-like: a New Form of Presentation of an Old Entity","updated":"2025-10-03T07:17:10+00:00","published_date":"2025-09-30","best_oa_location":{"url":"https://medicaljournalssweden.se/actadv/article/download/44373/51593","pmh_id":null,"url_for_pdf":"https://medicaljournalssweden.se/actadv/article/download/44373/51593","url_for_landing_page":"https://doi.org/10.2340/actadv.v105.44373"}}},"created_at":"2025-10-05T07:10:27.601Z","updated_at":"2025-10-06T07:08:34.463Z","tags":[{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"age_group","value":"adolescent","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"category","value":"Dermatology","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"condition","value":"Chronic Graft-Versus-Host Disease","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"generic","value":"methylprednisolone","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"generic","value":"ruxolitinib","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"modality","value":"Extracorporeal Photopheresis","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"modality","value":"JAK Inhibitor","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"modality","value":"Systemic Corticosteroid","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"sample_size","value":"n=1","value_numeric":1},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"sex","value":"female","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"study_design","value":"Case Report","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"warning","value":"Causality between ruxolitinib dose change and eruption is inferred, not proven.","value_numeric":null},{"paper_doi":"10.2340/actadv.v105.44373","tag_type":"warning","value":"Single-case short communication; novelty claim and generalizability are limited.","value_numeric":null}],"summaries":{"what_studied":"A single adolescent post–allogeneic HSCT with new-onset folliculocentric hyperkeratotic spicules resembling lichen spinulosus, assessed with skin biopsy and viral testing. The eruption arose after ruxolitinib was reduced from 2.5 mg BID to 2.5 mg QD and was tracked through subsequent treatment changes in 2023.","key_result":"Lesions appeared after ruxolitinib dose reduction and cleared after intensifying immunosuppression with oral methylprednisolone, extracorporeal photopheresis, and ruxolitinib. Biopsy showed compact parakeratotic hyperkeratosis at the follicular infundibulum with basal melanophagia; TSPyV immunohistochemistry was negative and immunofixation was nonspecific.","limitations":"This is a single-case observation, so generalizability is limited. The attribution to cGVHD relies on clinicopathologic correlation and temporal association with immunosuppression changes rather than a definitive diagnostic test.","practice_takeaway":"In pediatric HSCT recipients, spiny folliculocentric eruptions mimicking lichen spinulosus can signal cutaneous cGVHD; exclude trichodysplasia spinulosa with histology and TSPyV testing. Such lesions may improve with escalation of immunosuppression, including systemic corticosteroids, extracorporeal photopheresis, and JAK inhibitor adjustment."}}],"initialOffset":15,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"generic","value":"methylprednisolone"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Generics","href":"/hub/tag/generic"},{"name":"methylprednisolone"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"generic","hub_tag_value":"methylprednisolone"}}}]]

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