2f:I[703058,["/_next/static/immutable/chunks/3bhq_5eh1d760.js","/_next/static/immutable/chunks/1855-ogyi8eh4.js","/_next/static/immutable/chunks/2pfsjpn6k6zlg.js","/_next/static/immutable/chunks/26c7v3_3uwnwu.js","/_next/static/immutable/chunks/25a3ozcrvi1ff.js","/_next/static/immutable/chunks/2r4wx50nvjh_4.js","/_next/static/immutable/chunks/23fsw06hsi5b1.js","/_next/static/immutable/chunks/06_yt270h41rg.js","/_next/static/immutable/chunks/3ezsiz-7shkna.js","/_next/static/immutable/chunks/20x2y96ldu5wx.js","/_next/static/immutable/chunks/2lxntcm3pd1_h.js","/_next/static/immutable/chunks/00dgco8irq3jw.js","/_next/static/immutable/chunks/00nwe-kq0rboc.js","/_next/static/immutable/chunks/2051ffx3xzio9.js"],"PaperListPageClient"] 30:T6eb,The aim of this study was to compare differences in clinical response, drug survival, and adverse event rates between anakinra and canakinumab in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. This multicenter international study includes patients with VEXAS from France, Israel, and Italy treated with interleukin-1 inhibition. Global response (GR) was defined as the absence of inflammatory symptoms and ≥50% decrease in steroid dose and C-reactive protein. Multiple regression analysis was performed to identify associated variables. Drug survival was analyzed using Kaplan-Meier plots and log-rank test, with Cox regression models for associated factors. We included 47 male patients with VEXAS; 44 received anakinra and 9 received canakinumab, with 6 patients using both at different time points. GR at 1 month was 34% for anakinra and 100% for canakinumab (P < 0.001) and 22% and 78% at 3 months, respectively (P = 0.001). Treatment with canakinumab was associated with a higher odds ratio (OR) of achieving GR at 3 months (OR 28.8, 95% confidence interval 3.0-273.9; P = 0.004) in a multivariable analysis. Median drug survival was 54 (interquartile range [IQR] 30-56) months for canakinumab at 300 mg/month compared with 7 (IQR 4-8) months for canakinumab 150 mg/month and 1 (IQR 1-2.5) months for anakinra (P = 0.01). Injection-site reactions were only recorded for the anakinra group (47 vs 0%; P = 0.006), whereas infections were more frequent in the anakinra group (31% and 11%; P = 0.3). Canakinumab demonstrated superior clinical response and drug survival with fewer adverse events compared with anakinra. Monthly canakinumab 300 mg may be considered as an effective steroid-sparing therapeutic option for patients with VEXAS.31:Td08, Objective The aim of this study was to compare differences in clinical response, drug survival, and adverse event rates between anakinra and canakinumab in VEXAS (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) syndrome. Methods This multicenter international study includes patients with VEXAS from France, Israel, and Italy treated with interleukin‐1 inhibition. Global response (GR) was defined as the absence of inflammatory symptoms and ≥50% decrease in steroid dose and C‐reactive protein. Multiple regression analysis was performed to identify associated variables. Drug survival was analyzed using Kaplan‐Meier plots and log‐rank test, with Cox regression models for associated factors. Results We included 47 male patients with VEXAS; 44 received anakinra and 9 received canakinumab, with 6 patients using both at different time points. GR at 1 month was 34% for anakinra and 100% for canakinumab ( P < 0.001) and 22% and 78% at 3 months, respectively ( P = 0.001). Treatment with canakinumab was associated with a higher odds ratio (OR) of achieving GR at 3 months (OR 28.8, 95% confidence interval 3.0–273.9; P = 0.004) in a multivariable analysis. Median drug survival was 54 (interquartile range [IQR] 30–56) months for canakinumab at 300 mg/month compared with 7 (IQR 4–8) months for canakinumab 150 mg/month and 1 (IQR 1–2.5) months for anakinra ( P = 0.01). Injection‐site reactions were only recorded for the anakinra group (47 vs 0%; P = 0.006), whereas infections were more frequent in the anakinra group (31% and 11%; P = 0.3). Conclusion Canakinumab demonstrated superior clinical response and drug survival with fewer adverse events compared with anakinra. Monthly canakinumab 300 mg may be considered as an effective steroid‐sparing therapeutic option for patients with VEXAS.

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32:T43c,Among the 112 patients (median [range] age, 69 [39-79] years; 111 [99%] male), skin involvement was common (93 [83%]), and the most frequent presenting feature of disease (68 [61%]). Of 64 histopathologic reports available from 60 patients, predominant skin histopathologic findings were leukocytoclastic vasculitis (23 [36%]), neutrophilic dermatosis (22 [34%]), and perivascular dermatitis (19 [30%]). Distinct pathogenic genetic variants were associated with specific cutaneous manifestations. The p.Met41Leu variant was most frequently associated with neutrophilic dermal infiltrates (14 of 17 patients [82%]), often resembling histiocytoid Sweet syndrome. In contrast, the p.Met41Val variant was associated with vasculitic lesions (11 of 20 patients [55%]) with a mixed leukocytic infiltrate (17 of 20 patients [85%]). Oral prednisone improved skin manifestations in 67 of 73 patients (92%). Patients with VEXAS treated with anakinra frequently developed severe injection-site reactions (12 of 16 [75%]), including ulceration (2 of 12 [17%]) and abscess formation (1 of 12 [8%]).33:T946,VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined genetic disease with an estimated prevalence of 1 in 4269 men older than 50 years and is marked by systemic inflammation, progressive bone marrow failure, and inflammatory cutaneous manifestations. To define the spectrum of cutaneous manifestations in VEXAS syndrome and the association of these findings with clinical, genetic, and histological features. This observational cohort study included data from 112 patients who were diagnosed with VEXAS-defining genetic variants in UBA1 between 2019 and 2023. Data were collected from medical record review or from patients with VEXAS directly evaluated at the National Institutes of Health in Bethesda, Maryland. To define the spectrum of cutaneous manifestations in VEXAS in association with genetic, histological, and other clinical findings. A secondary outcome was cutaneous response to treatment in VEXAS. Among the 112 patients (median [range] age, 69 [39-79] years; 111 [99%] male), skin involvement was common (93 [83%]), and the most frequent presenting feature of disease (68 [61%]). Of 64 histopathologic reports available from 60 patients, predominant skin histopathologic findings were leukocytoclastic vasculitis (23 [36%]), neutrophilic dermatosis (22 [34%]), and perivascular dermatitis (19 [30%]). Distinct pathogenic genetic variants were associated with specific cutaneous manifestations. The p.Met41Leu variant was most frequently associated with neutrophilic dermal infiltrates (14 of 17 patients [82%]), often resembling histiocytoid Sweet syndrome. In contrast, the p.Met41Val variant was associated with vasculitic lesions (11 of 20 patients [55%]) with a mixed leukocytic infiltrate (17 of 20 patients [85%]). Oral prednisone improved skin manifestations in 67 of 73 patients (92%). Patients with VEXAS treated with anakinra frequently developed severe injection-site reactions (12 of 16 [75%]), including ulceration (2 of 12 [17%]) and abscess formation (1 of 12 [8%]). Results of this cohort study show that skin manifestations are a common and early manifestation of VEXAS syndrome. Genetic evaluation for VEXAS should be considered in older male patients with cutaneous vasculitis, neutrophilic dermatoses, or chondritis. Awareness of VEXAS among dermatologists is critical to facilitate early diagnosis.34:Tbbc,ImportanceVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined genetic disease with an estimated prevalence of 1 in 4269 men older than 50 years and is marked by systemic inflammation, progressive bone marrow failure, and inflammatory cutaneous manifestations.ObjectiveTo define the spectrum of cutaneous manifestations in VEXAS syndrome and the association of these findings with clinical, genetic, and histological features.Design, Setting, and ParticipantsThis observational cohort study included data from 112 patients who were diagnosed with VEXAS-defining genetic variants in UBA1 between 2019 and 2023. Data were collected from medical record review or from patients with VEXAS directly evaluated at the National Institutes of Health in Bethesda, Maryland.Main Outcomes and MeasuresTo define the spectrum of cutaneous manifestations in VEXAS in association with genetic, histological, and other clinical findings. A secondary outcome was cutaneous response to treatment in VEXAS.ResultsAmong the 112 patients (median [range] age, 69 [39-79] years; 111 [99%] male), skin involvement was common (93 [83%]), and the most frequent presenting feature of disease (68 [61%]). Of 64 histopathologic reports available from 60 patients, predominant skin histopathologic findings were leukocytoclastic vasculitis (23 [36%]), neutrophilic dermatosis (22 [34%]), and perivascular dermatitis (19 [30%]). Distinct pathogenic genetic variants were associated with specific cutaneous manifestations. The p.Met41Leu variant was most frequently associated with neutrophilic dermal infiltrates (14 of 17 patients [82%]), often resembling histiocytoid Sweet syndrome. In contrast, the p.Met41Val variant was associated with vasculitic lesions (11 of 20 patients [55%]) with a mixed leukocytic infiltrate (17 of 20 patients [85%]). Oral prednisone improved skin manifestations in 67 of 73 patients (92%). Patients with VEXAS treated with anakinra frequently developed severe injection-site reactions (12 of 16 [75%]), including ulceration (2 of 12 [17%]) and abscess formation (1 of 12 [8%]).Conclusions and RelevanceResults of this cohort study show that skin manifestations are a common and early manifestation of VEXAS syndrome. Genetic evaluation for VEXAS should be considered in older male patients with cutaneous vasculitis, neutrophilic dermatoses, or chondritis. Awareness of VEXAS among dermatologists is critical to facilitate early diagnosis.25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"VEXAS Syndrome: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on VEXAS Syndrome (condition). Clinician-written summaries and actionable practice insights. 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Clinicians should verify sex distribution in the full paper.","value_numeric":null}],"summaries":{"what_studied":"This study examined genotype-specific inflammatory mechanisms in VEXAS syndrome (caused by somatic UBA1 mutations) using longitudinal whole-blood and single-cell RNA-seq from 13 patients (79 samples) and human monocytic cell lines engineered to carry each major UBA1 variant (p.Met41Val, p.Met41Thr, p.Met41Leu).","key_result":"RNASE1 was the strongest correlate of longitudinal disease activity (r = 0.70, FDR < 0.05) and was elevated in patient monocytes. Ubiquitination defects, unfolded protein response, pro-inflammatory cytokine production, cell death, and RNASE1 upregulation followed a graded severity pattern (p.Met41Val > p.Met41Thr > p.Met41Leu). RIPK3 inhibition markedly attenuated all these pathological features across genotypes.","limitations":"Small patient cohort (n = 13), limiting statistical power and generalizability. Cell-line models may not fully replicate primary patient hematopoietic cell biology. Findings are preclinical; no RIPK3 inhibitor has been tested in VEXAS patients.","practice_takeaway":"RNASE1 may serve as a genotype-sensitive biomarker of VEXAS disease activity worth tracking longitudinally. RIPK3 inhibition is a promising precision therapeutic target in VEXAS, particularly for severe UBA1 genotypes, but requires clinical validation."}},{"doi":"10.1002/art.70282","pmid":"42529936","title":"Redefining the Landscape: Acquired Mutations Driving Systemic Autoinflammatory Diseases","journal_title":"Arthritis & Rheumatology","journal_issn":"2326-5191","published_at":"2026-07-30T00:00:00.000Z","url":"https://doi.org/10.1002/art.70282","source_score":5,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-05T13:22:45.850Z","updated_at":"2026-08-05T13:22:45.850Z","tags":[{"paper_doi":"10.1002/art.70282","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"AA Amyloidosis","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"Blau Syndrome","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"Cryopyrin-Associated Periodic Syndrome","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"NLRC4 Inflammasomopathy","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"Systemic Autoinflammatory Diseases","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"TNF Receptor-Associated Periodic Syndrome","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"modality","value":"Next-Generation Sequencing","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"sample_size","value":"n=5530","value_numeric":5530},{"paper_doi":"10.1002/art.70282","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"warning","value":"Exact age breakdown and sex distribution are not reported in the abstract, so age_group and sex tags are assigned 'mixed' by inference.","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"warning","value":"Study design is described as a cohort but involves retrospective chart review; level of evidence is assigned as 3 given the single-centre referral population.","value_numeric":null},{"paper_doi":"10.1002/art.70282","tag_type":"warning","value":"The first reported case of vertical (gonosomal) transmission of TRAPS is described here, but details are limited to the abstract — clinical confirmation and generalisability are uncertain.","value_numeric":null}],"summaries":{"what_studied":"This single-centre cohort study evaluated the prevalence, spectrum, and clinical impact of acquired (somatic mosaic) variants in systemic autoinflammatory diseases (SAIDs) among 5,530 patients referred between 2017–2025, using deep next-generation sequencing of an autoinflammatory gene panel.","key_result":"SAIDs were diagnosed in 403/5,530 patients (7.3%); mosaic variants accounted for 20.6% of SAIDs diagnoses (83/403). VEXAS made up 69% of mosaic cases, CAPS 24%, TRAPS 5%, Blau syndrome 1%, and NLRC4 inflammasomopathies 1%. Eight variants had a minor allele frequency ≤5%, and AA amyloidosis complicated 20% of late-onset mosaic CAPS cases.","limitations":"Single-centre design limits generalisability. Referral bias likely enriches for complex or late-onset cases. Clinical correlation relied on electronic medical records, which may be incomplete.","practice_takeaway":"Use deep next-generation sequencing panels capable of detecting low-level mosaicism (≤5% allele frequency) when evaluating adults with late-onset SAIDs — standard sequencing will miss these. Screen mosaic CAPS patients closely for AA amyloidosis, which occurred in 1 in 5 late-onset cases."}},{"doi":"10.1016/j.clindermatol.2026.07.026","pmid":"42508548","title":"Neutrophilic Dermatoses","journal_title":"Clinics in Dermatology","journal_issn":"0738-081X","published_at":"2026-07-27T00:00:00.000Z","url":"https://doi.org/10.1016/j.clindermatol.2026.07.026","source_score":1,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:17:49.924Z","updated_at":"2026-08-04T18:17:49.924Z","tags":[{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"category","value":"Dermatology","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Bazex Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Behçet Disease","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Cutaneous Vasculitis","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Erythema Elevatum Diutinum","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Neutrophilic Dermatoses","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Neutrophilic Eccrine Hidradenitis","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Paraneoplastic Dermatosis","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Pyoderma Gangrenosum","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Schnitzler Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Subcorneal Pustular Dermatosis","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"Sweet Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"warning","value":"Bazex syndrome in this context refers to the paraneoplastic acrokeratosis variant, not Bazex-Dupré-Christol syndrome — clinicians should confirm which is meant in clinical contexts.","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"warning","value":"No sample size applicable — this is a narrative review with no original patient cohort.","value_numeric":null},{"paper_doi":"10.1016/j.clindermatol.2026.07.026","tag_type":"warning","value":"The paper covers a broad, heterogeneous group of conditions; evidence quality likely varies considerably across individual disorders discussed.","value_numeric":null}],"summaries":{"what_studied":"This review evaluates the clinical and histopathologic features of neutrophilic dermatoses — including Sweet syndrome, pyoderma gangrenosum, VEXAS syndrome, and others — as cutaneous manifestations of underlying malignancy, along with current treatment options.","key_result":"Multiple neutrophilic dermatoses (e.g., Sweet syndrome, pyoderma gangrenosum, neutrophilic eccrine hidradenitis, subcorneal pustular dermatosis, erythema elevatum diutinum, Behçet's disease, Bazex syndrome, VEXAS syndrome) can serve as cutaneous markers of occult or overt malignancy, and may precede the cancer diagnosis.","limitations":"Narrative review without systematic methodology or meta-analysis; no original patient data or quantitative outcomes reported; conditions reviewed are rare and heterogeneous, limiting generalizable conclusions.","practice_takeaway":"When a patient presents with a sterile neutrophilic dermatosis, consider workup for an underlying malignancy — especially hematologic — as the skin finding may precede the cancer diagnosis. Early recognition can prompt timely systemic evaluation and improve outcomes."}},{"doi":"10.1002/acr.80117","pmid":"42439317","title":"Development of a Disease Activity Index for the Assessment of\n VEXAS\n Syndrome (\n VEXAS\n ‐\n DAI\n )","journal_title":"Arthritis Care & Research","journal_issn":"2151-464X","published_at":"2026-07-13T00:00:00.000Z","url":"https://doi.org/10.1002/acr.80117","source_score":4,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-19T13:39:52.755Z","updated_at":"2026-07-19T13:39:52.755Z","tags":[{"paper_doi":"10.1002/acr.80117","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"modality","value":"Disease Activity Assessment","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"sample_size","value":"n=158","value_numeric":158},{"paper_doi":"10.1002/acr.80117","tag_type":"study_design","value":"Consensus Development (Modified Delphi)","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"warning","value":"Prospective validation of the VEXAS-DAI is stated as ongoing; the instrument is not yet validated for use as a clinical trial endpoint.","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"warning","value":"Sample size of 158 refers to test cases used for scoring development, not enrolled patients — no patient-level clinical cohort was described.","value_numeric":null},{"paper_doi":"10.1002/acr.80117","tag_type":"warning","value":"Study design is expert consensus (Delphi); the level of evidence reflects methodological limitations inherent to this approach.","value_numeric":null}],"summaries":{"what_studied":"Development of a disease activity index (VEXAS-DAI) to quantify active inflammation across organ systems in patients with VEXAS syndrome, using modified Delphi methodology with a multinational expert advisory committee.","key_result":"Consensus (≥75% concurrence) was reached after 4 Delphi rounds. The VEXAS-DAI covers 12 domains (inflammatory rash, chondritis, periorbital, ophthalmologic, joint, pulmonary, cardiovascular, genitourinary, neurological, oral/GI, renal, and constitutional symptoms) with a total score of 0–40; item grading was adapted from CTCAE criteria and scoring was developed using 158 test cases.","limitations":"The VEXAS-DAI has not yet been prospectively validated; consensus-based development relies on expert opinion rather than empirical patient data; the index was built for an emerging disease with limited longitudinal outcome evidence.","practice_takeaway":"The VEXAS-DAI provides the first structured tool to track inflammatory disease activity over time in VEXAS syndrome, which can support enrollment and outcome assessment in future clinical trials. Clinicians should await prospective validation before using it as a primary endpoint in research or routine care."}},{"doi":"10.1038/s41584-026-01407-0","pmid":"42410236","title":"Dysfunctional UBA1-mutated myeloid cells promote inflammation in VEXAS syndrome","journal_title":"Nature Reviews Rheumatology","journal_issn":"1759-4790","published_at":"2026-07-06T00:00:00.000Z","url":"https://doi.org/10.1038/s41584-026-01407-0","source_score":7,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-13T17:42:39.040Z","updated_at":"2026-07-13T17:42:39.040Z","tags":[{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"condition","value":"Autoinflammatory Disease","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"generic","value":"UBA1","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"modality","value":"Ubiquitin-Proteasome Pathway Modulation","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"study_design","value":"Review Article","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"warning","value":"Study design classified as Review Article based on journal section (Nature Reviews Rheumatology) and title framing; confirmation from full text is recommended.","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"warning","value":"The paper was published July 6, 2026 and is very recent; full text was not provided, so summary content is derived solely from the title, journal context, and available metadata. Specific findings, figures, and quantitative results could not be extracted.","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01407-0","tag_type":"warning","value":"UBA1 is listed as a generic tag as it is a gene/enzyme target rather than a drug; no pharmacologic agent was identified in the available metadata.","value_numeric":null}],"summaries":{"what_studied":"This paper examines how UBA1-mutated myeloid cells drive inflammation in VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome, focusing on the pathogenic mechanisms by which dysfunctional ubiquitylation in myeloid cells promotes inflammatory signaling.","limitations":"No primary patient data or clinical trial results are reported; conclusions are based on review and synthesis of existing literature rather than original experimental findings.","practice_takeaway":"Clinicians managing VEXAS syndrome should recognize that myeloid cell dysfunction driven by somatic UBA1 mutations is central to its autoinflammatory phenotype, which may guide future targeting of the ubiquitin-proteasome pathway in treatment strategies."}},{"doi":"10.1002/ajh.70401","pmid":"42260942","title":"Therapeutic Outcomes in\n VEXAS\n Syndrome: A Multicenter Comparative Cohort of Allogeneic Hematopoietic Stem Cell Transplantation and Hypomethylating Agents","journal_title":"American Journal of Hematology","journal_issn":"0361-8609","published_at":"2026-06-08T00:00:00.000Z","url":"https://doi.org/10.1002/ajh.70401","source_score":9,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-12T13:24:26.614Z","updated_at":"2026-06-12T13:24:26.614Z","tags":[{"paper_doi":"10.1002/ajh.70401","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"category","value":"Hematology","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"generic","value":"azacitidine","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"modality","value":"Allogeneic Hematopoietic Stem Cell Transplantation","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"modality","value":"Hypomethylating Agent","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"sample_size","value":"n=66","value_numeric":66},{"paper_doi":"10.1002/ajh.70401","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"warning","value":"Molecular remission denominator for alloHSCT is described as 'all evaluable patients,' which may not equal the full cohort of 31 — exact evaluable n is not specified in the abstract.","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"warning","value":"Specific HMA agents used (e.g., azacitidine vs. decitabine) are not detailed in the abstract; the generic tag 'azacitidine' is an inference based on common practice in VEXAS.","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"warning","value":"The HMA group was older on average than the alloHSCT group — age imbalance may persist as a confounder despite multivariable adjustment and sensitivity analyses.","value_numeric":null},{"paper_doi":"10.1002/ajh.70401","tag_type":"warning","value":"The 'impact_flag: practice_changing' tag reflects the first multicenter comparative survival data for alloHSCT vs. HMA in VEXAS, but prospective validation is explicitly called for by the authors.","value_numeric":null}],"summaries":{"what_studied":"This multicenter retrospective cohort study compared allogeneic hematopoietic stem cell transplantation (alloHSCT, n=31) versus hypomethylating agents (HMA, n=35) in 66 patients with VEXAS syndrome, with a median follow-up of 18 months from therapy initiation.","key_result":"AlloHSCT achieved molecular remission in 100% of evaluable patients and glucocorticoid discontinuation in 58%, versus 22% molecular remission and 6% glucocorticoid discontinuation with HMA. On multivariable analysis, alloHSCT was associated with significantly improved overall survival (HR=0.20, 95% CI: 0.05–0.81; p=0.024), with 3 deaths in the alloHSCT group vs. 11 in the HMA group.","limitations":"- Retrospective design limits causal inference.\n- HMA group was older on average, which may bias survival comparisons despite multivariable adjustment.\n- Small sample size (n=66) restricts statistical power and generalizability.","practice_takeaway":"In eligible patients with VEXAS syndrome, alloHSCT appears to offer superior molecular remission rates and a meaningful survival advantage over HMA — consider it early as a potentially curative strategy. HMA carries a high real-world discontinuation rate due to toxicity or lack of response and should be viewed as a bridge or option for transplant-ineligible patients."}},{"doi":"10.1016/j.berh.2026.102164","pmid":"42248698","title":"Pneumocystis prophylaxis in rheumatic disease","journal_title":"Best Practice & Research Clinical Rheumatology","journal_issn":"1521-6942","published_at":"2026-06-05T00:00:00.000Z","url":"https://doi.org/10.1016/j.berh.2026.102164","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-10T14:24:12.738Z","updated_at":"2026-06-10T14:24:12.738Z","tags":[{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"ANCA-Associated Vasculitis","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"Dermatomyositis","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"Giant Cell Arteritis","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"Pneumocystis Jirovecii Pneumonia","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"Rheumatic Disease","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"generic","value":"trimethoprim-sulfamethoxazole","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"modality","value":"Antibiotic Prophylaxis","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"modality","value":"Glucocorticoid","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"warning","value":"No sample size is reported; this is a review article without a primary patient cohort.","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"warning","value":"Study design tagged as 'Narrative Review' based on abstract language ('this review examines'); formal systematic review methodology is not confirmed.","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"warning","value":"The anti-MDA5 antigen is referenced in the abstract as 'anti-MD5' which appears to be a typo for anti-MDA5 (melanoma differentiation-associated protein 5); interpreted accordingly.","value_numeric":null},{"paper_doi":"10.1016/j.berh.2026.102164","tag_type":"warning","value":"Trimethoprim-sulfamethoxazole is inferred as the primary prophylaxis agent based on general clinical context; the abstract does not explicitly name the agent used.","value_numeric":null}],"summaries":{"what_studied":"This review evaluates PJP prophylaxis in rheumatic diseases, examining efficacy, adverse effects, and risk factors across conditions to support a risk-stratified, individualized approach to prophylaxis decisions.","key_result":"Routine PJP prophylaxis is warranted in high-risk conditions (ANCA-associated vasculitis during induction, anti-MDA5+ dermatomyositis, VEXAS syndrome) especially with high-dose glucocorticoids, while low-risk conditions like Giant Cell Arteritis do not justify routine prophylaxis. PJP mortality in rheumatic disease exceeds that seen in HIV-infected patients.","limitations":"Evidence-based guidelines specific to rheumatic disease are lacking; the review is narrative in design without systematic pooled data or meta-analysis, limiting quantitative conclusions.","practice_takeaway":"Use a risk-stratified framework: prioritize PJP prophylaxis for patients with ANCA-associated vasculitis on induction therapy, anti-MDA5+ dermatomyositis, or VEXAS syndrome receiving high-dose glucocorticoids. Routine prophylaxis is not needed for all rheumatic disease patients — tailor decisions to individual risk."}},{"doi":"10.1016/j.kint.2026.02.042","pmid":"42119779","title":"Tubulointerstitial Diseases: An Updated Framework for Diverse and Emerging Entities","journal_title":"Kidney International","journal_issn":"0085-2538","published_at":"2026-05-12T00:00:00.000Z","url":"https://doi.org/10.1016/j.kint.2026.02.042","source_score":15,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-15T13:23:05.936Z","updated_at":"2026-05-15T13:23:05.936Z","tags":[{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"category","value":"Urology & Nephrology","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"condition","value":"IgG4-Related Tubulointerstitial Nephritis","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"condition","value":"IgM Plasma Cell Tubulointerstitial Nephritis","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"condition","value":"Immune Checkpoint Inhibitor-Associated Nephritis","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"condition","value":"Tubulointerstitial Nephritis","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"condition","value":"VEXAS Syndrome","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"level_of_evidence","value":"6","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"modality","value":"Immune Checkpoint Inhibitor","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"warning","value":"No original patient data are presented; all conclusions are based on expert synthesis and literature review. Evidence levels for individual TIN entities vary widely and are not formally graded within the paper.","value_numeric":null},{"paper_doi":"10.1016/j.kint.2026.02.042","tag_type":"warning","value":"The eight-category framework is proposed by the authors and has not yet been validated in prospective cohorts or adopted by major nephrology societies as of the publication date.","value_numeric":null}],"summaries":{"what_studied":"This review proposes an updated etiologic framework for tubulointerstitial nephritis/nephropathy (TIN), organizing diverse and emerging entities into eight categories: drug effect, autoimmune/immune-mediated, infection-associated, hereditary/genetic, toxic/metabolic, monoclonal protein-associated, mimics of TIN, and idiopathic/other.","key_result":"The review highlights recently characterized TIN entities — including immune checkpoint inhibitor-associated TIN, IgG4-related TIN, IgM plasma cell TIN, and VEXAS syndrome — and proposes that IgG4-related TIN serves as a model for how new entities are recognized and classified.","limitations":"As a narrative review, no original patient data or comparative outcomes are reported; the framework is consensus- and expert opinion-based rather than derived from prospective or systematic evidence.","practice_takeaway":"Clinicians should apply the eight-category etiologic framework when evaluating TIN to improve diagnostic precision and guide targeted therapy. Emerging entities like immune checkpoint inhibitor-associated TIN and VEXAS syndrome should be actively considered in the differential, especially as novel therapeutics become more common."}},{"doi":"10.1002/art.43384","pmid":"40977434","title":"Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With\n VEXAS\n Syndrome: An International Multicenter Study","journal_title":"Arthritis & Rheumatology","journal_issn":"2326-5191","published_at":"2025-09-22T00:00:00.000Z","url":"https://doi.org/10.1002/art.43384","source_score":37,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-01-07","doi":"10.1002/art.43384","days":"30","issn":"2326-5191","rank":"2","field":"Clinical Medicine","score":"37","category":"Rheumatology","categories":["Rheumatology"],"_categories":["Rheumatology"]},"pubmed":{"doi":"10.1002/art.43384","title":"Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With VEXAS Syndrome: An International Multicenter Study.","journal":"Arthritis & rheumatology (Hoboken, N.J.)","methods":"","results":"We included 47 male patients with VEXAS; 44 received anakinra and 9 received canakinumab, with 6 patients using both at different time points. GR at 1 month was 34% for anakinra and 100% for canakinumab (P < 0.001) and 22% and 78% at 3 months, respectively (P = 0.001). Treatment with canakinumab was associated with a higher odds ratio (OR) of achieving GR at 3 months (OR 28.8, 95% confidence interval 3.0-273.9; P = 0.004) in a multivariable analysis. Median drug survival was 54 (interquartile range [IQR] 30-56) months for canakinumab at 300 mg/month compared with 7 (IQR 4-8) months for canakinumab 150 mg/month and 1 (IQR 1-2.5) months for anakinra (P = 0.01). Injection-site reactions were only recorded for the anakinra group (47 vs 0%; P = 0.006), whereas infections were more frequent in the anakinra group (31% and 11%; P = 0.3).","abstract":"$30","keywords":[],"pubmed_id":"40977434","conclusions":"Canakinumab demonstrated superior clinical response and drug survival with fewer adverse events compared with anakinra. Monthly canakinumab 300 mg may be considered as an effective steroid-sparing therapeutic option for patients with VEXAS.","publication_date":"2025-09-22T00:00:00"},"crossref":{"DOI":"10.1002/art.43384","URL":"https://doi.org/10.1002/art.43384","ISSN":["2326-5191","2326-5205"],"link":[{"URL":"https://acrjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/art.43384","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":null,"type":"journal-article","issue":null,"score":1,"title":["Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With\n VEXAS\n Syndrome: An International Multicenter Study"],"author":[{"given":"Tali","family":"Eviatar","sequence":"first","affiliation":[{"name":"Rheumatology Department Tel Aviv Medical Center Tel Aviv Israel"},{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"}]},{"given":"Dafne","family":"Capelusnik","sequence":"additional","affiliation":[{"name":"Rheumatology Department Tel Aviv Medical Center Tel Aviv Israel"},{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"},{"name":"Care and Public Health Research Institute Maastricht University Maastricht the Netherlands"}]},{"given":"Corrado","family":"Campochiaro","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases San Raffaele Scientific Institute Milan Italy"}]},{"given":"Valentin","family":"Lacombe","sequence":"additional","affiliation":[{"name":"Médecine Interne et Polyvalente Centre Hospitalier du Haut‐Anjou Château‐Gontier France"},{"name":"Médecine Interne et Immunologie Clinique Centre Hospitalier Universitaire d'Angers Angers France"}]},{"given":"Vincent","family":"Jachiet","sequence":"additional","affiliation":[{"name":"Sorbonne Université, Service de Médecine Interne, Hôpital Saint‐Antoine, CEREMAIAA, AP‐HP Paris France"}]},{"given":"Michael","family":"Zisapel","sequence":"additional","affiliation":[{"name":"Rheumatology Department Tel Aviv Medical Center Tel Aviv Israel"},{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"}]},{"given":"Iftach","family":"Sagy","sequence":"additional","affiliation":[{"name":"Soroka Medical Center Rheumatic Disease Unit Be'er Sheva Israel"},{"name":"Clinical Research Center Soroka Medical Center Be'er Sheva Israel"}]},{"given":"Oshrat E.","family":"Tayer‐Shifman","sequence":"additional","affiliation":[{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"},{"name":"Meir Medical Center, Rheumatology Unit Kfar Saba Israel"}]},{"given":"David","family":"Ozeri","sequence":"additional","affiliation":[{"name":"Sheba Medical Center, Rheumatology Unit Tel HaShomer Israel"}]},{"given":"Shaye","family":"Kivity","sequence":"additional","affiliation":[{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"},{"name":"Meir Medical Center, Rheumatology Unit Kfar Saba Israel"}]},{"given":"Alessandro","family":"Tomelleri","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases San Raffaele Scientific Institute Milan Italy"}]},{"given":"Benjamin","family":"Terrier","sequence":"additional","affiliation":[{"name":"Médecine Interne, Hôpital Cochin, AP‐HP, Université Paris Cité Paris France"}]},{"given":"Hagit","family":"Peleg","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Hadassah University Medical Center Jerusalem Israel"},{"name":"Internal Medicine Department, Hadassah University Medical Center Jerusalem Israel"}]},{"given":"Thibault","family":"Comont","sequence":"additional","affiliation":[{"name":"Service de Médecine Interne, Institut Universitaire Du Cancer De Toulouse Oncopole, Centre Hospitalier Universitaire de Toulouse, Université Paul Sabatier Toulouse France"}]},{"given":"Karim","family":"Sacre","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Bichat‐Claude Bernard Hospital Paris France"}]},{"given":"Pascal","family":"Woaye‐Hune","sequence":"additional","affiliation":[{"name":"Service de Médecine Interne, Centre Hospitalier Départemental Vendée La Roche‐sur‐Yon France"}]},{"given":"Laurent","family":"Arnaud","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, National Reference Center for Rare Diseases, Hôpitaux Universitaires de Strasbourg, INSERM UMR‐S 1109 Strasbourg France"}]},{"given":"Estibaliz","family":"Lazaro","sequence":"additional","affiliation":[{"name":"Internal Medicine, Centre Hospitalier Universitaire de Bordeaux Bordeaux France"}]},{"given":"Vincent","family":"Grobost","sequence":"additional","affiliation":[{"name":"Médecine Interne, Centre Hospitalier Universitaire Clermont‐Ferrand Estaing Clermont‐Ferrand France"}]},{"given":"Francois","family":"Lifermann","sequence":"additional","affiliation":[{"name":"Service de Médecine Interne, Centre Hospitalier Dax Dax France"}]},{"given":"Maxime","family":"Samson","sequence":"additional","affiliation":[{"name":"Service de Médecine Interne et Immunologie Clinique, Centre de Référence Constitutif des Maladies Auto‐immunes et Auto‐inflammatoires Rares de l'adulte, Centre Hospitalier Universitaire Dijon‐Bourgogne Dijon France"},{"name":"Université de Bourgogne Franche‐Comté, INSERM, EFS BFC, UMR1098, RIGHT Interactions Greffon‐Hôte‐Tumeur/Ingénierie Cellulaire et Génique Besançon France"}]},{"given":"Samuel","family":"Ardois","sequence":"additional","affiliation":[{"name":"Médecine Interne, Centre Hospitalier Universitaire Rennes Rennes France"}]},{"given":"Alice","family":"Garnier","sequence":"additional","affiliation":[{"name":"Hematology Department, Nantes University Hospital Nantes France"}]},{"given":"Alexandre","family":"Maria","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine ‐ Multi‐organ Diseases, Saint Eloi Hospital, Montpellier University Hospital, University Montpellier Montpellier France"}]},{"given":"Alain","family":"Cantagrel","sequence":"additional","affiliation":[{"name":"Service de Rhumatologie, Hôpital Pierre‐Paul Riquet, Centre Hospitalier Universitaire de Toulouse Toulouse France"}]},{"given":"Aurore","family":"Meyer","sequence":"additional","affiliation":[{"name":"Service d'Immunologie Clinique et Médecine Interne, Hôpitaux Universitaires de Strasbourg Strasbourg France"}]},{"given":"Jean‐David","family":"Bouaziz","sequence":"additional","affiliation":[{"name":"Dermatology, Hôpital Saint‐Louis Paris France"}]},{"given":"Mael","family":"Heiblig","sequence":"additional","affiliation":[{"name":"Hématologie, Hôpital Lyon Sud, Hospices Civils de Lyon Pierre‐Bénite France"}]},{"given":"Lorenzo","family":"Dagna","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases San Raffaele Scientific Institute Milan Italy"}]},{"given":"Elisa","family":"Diral","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases San Raffaele Scientific Institute Milan Italy"}]},{"given":"Olivier","family":"Kosmider","sequence":"additional","affiliation":[{"name":"Université Paris Cité, Institut Cochin, INSERM U1016, CNRS UMR8104 AP‐HP, Laboratory of Hematology Hôpital Cochin Paris France"}]},{"given":"Ori","family":"Elkayam","sequence":"additional","affiliation":[{"name":"Rheumatology Department Tel Aviv Medical Center Tel Aviv Israel"},{"name":"Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"}]},{"given":"Jérôme","family":"Hadjadj","sequence":"additional","affiliation":[{"name":"Sorbonne Université, Service de Médecine Interne, Hôpital Saint‐Antoine, CEREMAIAA, AP‐HP Paris France"}]},{"given":"Sophie","family":"Georgin‐Lavialle","sequence":"additional","affiliation":[{"name":"Médecine Interne, CEREMAIA, Sorbonne Université, Hospital Tenon Paris France"}]},{"given":"Olivier","family":"Fain","sequence":"additional","affiliation":[{"name":"Sorbonne Université, Service de Médecine Interne, Hôpital Saint‐Antoine, CEREMAIAA, AP‐HP Paris France"}]},{"given":"Arsene","family":"Mekinian","sequence":"additional","affiliation":[{"name":"Sorbonne Université, Service de Médecine Interne, Hôpital Saint‐Antoine, CEREMAIAA, AP‐HP Paris 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Corticosteroid","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"sample_size","value":"n=112","value_numeric":112},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"study_design","value":"Observational Cohort Study","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"warning","value":"Histopathology findings are based on a subset (64 reports from 60 of 112 patients).","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"warning","value":"Observational cohort with mixed data sources (chart review vs NIH evaluations).","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2024.1657","tag_type":"warning","value":"Skin treatment response was assessed by physician impression, not standardized criteria.","value_numeric":null}],"summaries":{"what_studied":"An observational cohort of 112 patients with UBA1-variant–confirmed VEXAS (2019–2023) characterized the spectrum of skin findings and their associations with genotype, histology, and other clinical features; a secondary aim assessed skin response to treatments.","key_result":"Skin involvement occurred in 93/112 (83%) and was the presenting feature in 68/112 (61%); histopathology (64 reports from 60 patients) most often showed leukocytoclastic vasculitis 23/64 (36%), neutrophilic dermatosis 22/64 (34%), and perivascular dermatitis 19/64 (30%). Genotype–phenotype links included p.Met41Leu with neutrophilic infiltrates 14/17 (82%) and p.Met41Val with vasculitic lesions 11/20 (55%) and mixed leukocytic infiltrates 17/20 (85%); prednisone improved skin in 67/73 (92%), while anakinra caused severe injection-site reactions in 12/16 (75%) with ulceration 2/12 (17%) and abscess 1/12 (8%).","limitations":"Data came from mixed sources (retrospective chart review and NIH evaluations), histopathology was available for only 60/112 patients, and treatment response was based on physician impression rather than standardized measures.","practice_takeaway":"In older men with cutaneous vasculitis, neutrophilic dermatoses, or chondritis, consider testing for VEXAS (UBA1). Prednisone often improves skin disease, while anakinra may cause frequent severe injection-site reactions."}}],"initialOffset":10,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"VEXAS Syndrome"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"VEXAS Syndrome"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"VEXAS Syndrome"}}}]]

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