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:T4d8,Of the 90 patients in the Ritux 3 trial, 83 were evaluated at the end of follow-up study visit (44 in the rituximab plus prednisone group; 39 in the prednisone-alone group) with a median (IQR) follow-up of 87.3 (79.1-97.5) months. Forty-three patients (93%) from the rituximab plus prednisone and 17 patients (39%) from the prednisone-alone group had achieved complete remission without corticosteroids at any time during the follow-up. Patients from the rituximab group had much longer 5- and 7-year DFS without corticosteroids than patients from the prednisone-alone group (76.7% and 72.1% vs 35.3% and 35.3%, respectively; P < .001), and had about half the relapses (42.2% vs 83.7%; P < .001). Patients who received rituximab as second-line treatment had shorter DFS than patients treated as first line (P = .007). Fewer SAEs were reported in the rituximab plus prednisone group compared with the prednisone-alone group, 31 vs 58 respectively, corresponding to 0.67 and 1.32 SAEs per patient, respectively (P = .003). The combination of anti-Dsg1 values of 20 or more IU/mL and/or anti-Dsg3 values of 48 or more IU/mL yielded 0.83 positive predictive value and 0.94 negative predictive value to predict long-term relapse.31:T99c,The Ritux 3 trial demonstrated the short-term efficacy and safety of first-line treatment with rituximab compared with a standard corticosteroid regimen in pemphigus. No data on the long-term follow-up of patients who received rituximab as first line are available. To assess the long-term efficacy and safety of the Ritux 3 treatment regimen. This 7-year follow-up study of the Ritux 3 trial included patients with pemphigus from 25 dermatology departments in France from January 1, 2010, to December 31, 2015. Patients were initially randomized in the rituximab plus prednisone group or prednisone-alone group. The primary outcome was the 5- and 7-year disease-free survival (DFS) without corticosteroids, assessed by Kaplan-Meier curves. Secondary outcomes were occurrence of relapse, occurrence of severe adverse events (SAEs), and evolution of antidesmoglein (Dsg) antibody enzyme-linked immunosorbent assay values to predict long-term relapse. Of the 90 patients in the Ritux 3 trial, 83 were evaluated at the end of follow-up study visit (44 in the rituximab plus prednisone group; 39 in the prednisone-alone group) with a median (IQR) follow-up of 87.3 (79.1-97.5) months. Forty-three patients (93%) from the rituximab plus prednisone and 17 patients (39%) from the prednisone-alone group had achieved complete remission without corticosteroids at any time during the follow-up. Patients from the rituximab group had much longer 5- and 7-year DFS without corticosteroids than patients from the prednisone-alone group (76.7% and 72.1% vs 35.3% and 35.3%, respectively; P < .001), and had about half the relapses (42.2% vs 83.7%; P < .001). Patients who received rituximab as second-line treatment had shorter DFS than patients treated as first line (P = .007). Fewer SAEs were reported in the rituximab plus prednisone group compared with the prednisone-alone group, 31 vs 58 respectively, corresponding to 0.67 and 1.32 SAEs per patient, respectively (P = .003). The combination of anti-Dsg1 values of 20 or more IU/mL and/or anti-Dsg3 values of 48 or more IU/mL yielded 0.83 positive predictive value and 0.94 negative predictive value to predict long-term relapse. In this secondary analysis of the Ritux 3 trail, first-line treatment of patients with pemphigus with the Ritux 3 regimen was associated with long-term sustained complete remission without corticosteroid therapy without any additional maintenance infusion of rituximab.32:Tcd3,ImportanceThe Ritux 3 trial demonstrated the short-term efficacy and safety of first-line treatment with rituximab compared with a standard corticosteroid regimen in pemphigus. No data on the long-term follow-up of patients who received rituximab as first line are available.ObjectiveTo assess the long-term efficacy and safety of the Ritux 3 treatment regimen.Design, Setting, and ParticipantsThis 7-year follow-up study of the Ritux 3 trial included patients with pemphigus from 25 dermatology departments in France from January 1, 2010, to December 31, 2015.ExposurePatients were initially randomized in the rituximab plus prednisone group or prednisone-alone group.Main outcomes and measuresThe primary outcome was the 5- and 7-year disease-free survival (DFS) without corticosteroids, assessed by Kaplan-Meier curves. Secondary outcomes were occurrence of relapse, occurrence of severe adverse events (SAEs), and evolution of antidesmoglein (Dsg) antibody enzyme-linked immunosorbent assay values to predict long-term relapse.ResultsOf the 90 patients in the Ritux 3 trial, 83 were evaluated at the end of follow-up study visit (44 in the rituximab plus prednisone group; 39 in the prednisone-alone group) with a median (IQR) follow-up of 87.3 (79.1-97.5) months. Forty-three patients (93%) from the rituximab plus prednisone and 17 patients (39%) from the prednisone-alone group had achieved complete remission without corticosteroids at any time during the follow-up. Patients from the rituximab group had much longer 5- and 7-year DFS without corticosteroids than patients from the prednisone-alone group (76.7% and 72.1% vs 35.3% and 35.3%, respectively; P &lt; .001), and had about half the relapses (42.2% vs 83.7%; P &lt; .001). Patients who received rituximab as second-line treatment had shorter DFS than patients treated as first line (P = .007). Fewer SAEs were reported in the rituximab plus prednisone group compared with the prednisone-alone group, 31 vs 58 respectively, corresponding to 0.67 and 1.32 SAEs per patient, respectively (P = .003). The combination of anti-Dsg1 values of 20 or more IU/mL and/or anti-Dsg3 values of 48 or more IU/mL yielded 0.83 positive predictive value and 0.94 negative predictive value to predict long-term relapse.Conclusions and RelevanceIn this secondary analysis of the Ritux 3 trail, first-line treatment of patients with pemphigus with the Ritux 3 regimen was associated with long-term sustained complete remission without corticosteroid therapy without any additional maintenance infusion of rituximab.25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"Pemphigus: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on Pemphigus (condition). Clinician-written summaries and actionable practice insights. 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Key details such as sample size, clinical outcomes, and treatment data are not verifiable.","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2026.3186","tag_type":"warning","value":"Level of evidence set to 4 (case study/small series) based on context, but could not be confirmed from full text.","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2026.3186","tag_type":"warning","value":"The study design label 'Genetic Association Study' is taken directly from the abstract; the study may be a case report or case series given the context of re-examining a prior single case report.","value_numeric":null}],"summaries":{"what_studied":"This genetic association study re-examined a prior case report originally diagnosed as persistent neonatal pemphigus, evaluating whether the condition was autoimmune or genetic in origin, specifically investigating a homozygous pathogenic missense variant in *DSG3* (desmoglein-3) as the cause of acantholytic blistering of the oroesophageal mucosa.","key_result":"The study reclassified the prior case of persistent neonatal pemphigus as genetic rather than autoimmune in origin, attributing the oroesophageal acantholytic blistering to a homozygous *DSG3* pathogenic missense variant.","limitations":"Only an abstract is publicly available due to a publication embargo; full text is not accessible until September 2027. Sample size and detailed clinical data cannot be assessed. Study appears limited to a single case or very small cohort, restricting generalizability.","practice_takeaway":"In neonates or infants presenting with persistent mucosal blistering resembling pemphigus, consider genetic testing for *DSG3* variants before attributing disease to autoimmune mechanisms. A genetic etiology has fundamentally different treatment and counseling implications than autoimmune pemphigus."}},{"doi":"10.1001/jamadermatol.2023.5679","pmid":"38265821","title":"Sustained Remission Without Corticosteroids Among Patients With Pemphigus Who Had Rituximab as First-Line Therapy","journal_title":"JAMA Dermatology","journal_issn":"2168-6068","published_at":"2024-01-24T00:00:00.000Z","url":"https://www.ncbi.nlm.nih.gov/pmc/articles/10809134","source_score":41,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2024-02-19","doi":"10.1001/jamadermatol.2023.5679","days":"30","issn":"2168-6068","rank":"3","field":"Clinical Medicine","score":"41","category":"Dermatology","categories":["Dermatology"],"_categories":["Dermatology"]},"pubmed":{"doi":"10.1001/jamadermatol.2023.5679","title":"Sustained Remission Without Corticosteroids Among Patients With Pemphigus Who Had Rituximab as First-Line Therapy: Follow-Up of the Ritux 3 Trial.","journal":"JAMA 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Dermatology, CHU Rouen and INSERM U1234, Normandie University, Rouen, France"}]},{"given":"Estelle","family":"Houivet","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Clinical Research, CHU Rouen, Rouen, France"}]},{"given":"Claire","family":"Mignard","sequence":"additional","affiliation":[{"name":"Department of Dermatology, CHU Rouen and INSERM U1234, Normandie University, Rouen, France"}]},{"given":"Marie-Laure","family":"Golinski","sequence":"additional","affiliation":[{"name":"Department of Dermatology, CHU Rouen and INSERM U1234, Normandie University, Rouen, France"}]},{"given":"Sébastien","family":"Calbo","sequence":"additional","affiliation":[{"name":"Department of Dermatology, CHU Rouen and INSERM U1234, Normandie University, Rouen, France"}]},{"given":"Catherine","family":"Prost-Squarcioni","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Centre de référence des maladies bulleuses auto-immunes, Avicenne Hospital, Assistance Publique-Hôpitaux de Paris, University of Paris 13, Bobigny, France"}]},{"given":"Bruno","family":"Labeille","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Saint-Étienne, Saint-Étienne, France"}]},{"given":"Catherine","family":"Picard-Dahan","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Bichat Hospital, University of Paris 10, Paris, France"}]},{"given":"Guillaume","family":"Chaby","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Amiens, Amiens, France"}]},{"given":"Marie-Aleth","family":"Richard","sequence":"additional","affiliation":[{"name":"CEReSS-EA 3279, Research Centre in Health Services and Quality of Life Aix Marseille University, Dermatology Department, Universitaire Hospital Timone, Assistance Publique Hôpitaux de Marseille, APHM, 13385, Marseille, 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Accessed November 12, 2022. https://www.insee.fr/fr/information/4190491","article-title":null,"journal-title":null,"doi-asserted-by":null}],"short-title":[],"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3,1]]}},"content-domain":{"domain":[],"crossmark-restriction":false},"original-title":[],"container-title":["JAMA Dermatology"],"published-print":{"date-parts":[[2024,3,1]]},"reference-count":17,"published-online":null,"references-count":17,"short-container-title":["JAMA Dermatol"],"is-referenced-by-count":12},"unpaywall":{"doi":"10.1001/jamadermatol.2023.5679","is_oa":true,"title":"Sustained Remission Without Corticosteroids Among Patients With Pemphigus Who Had Rituximab as First-Line Therapy","updated":"2025-07-30T00:59:52+00:00","published_date":"2024-01-24","best_oa_location":{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/10809134","pmh_id":"oai:pubmedcentral.nih.gov:10809134","url_for_pdf":null,"url_for_landing_page":"https://www.ncbi.nlm.nih.gov/pmc/articles/10809134"}}},"created_at":"2025-09-20T08:52:49.368Z","updated_at":"2025-09-20T10:30:25.711Z","tags":[{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"category","value":"Dermatology","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"condition","value":"Pemphigus","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"generic","value":"rituximab","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"sample_size","value":"n=83","value_numeric":83},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"study_design","value":"Randomized Controlled Trial","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"warning","value":"Anti-desmoglein cutoff performance (PPV/NPV) was derived within this cohort and may not generalize.","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"warning","value":"Long-term follow-up phase occurred after initial randomization; the extension itself was not randomized.","value_numeric":null},{"paper_doi":"10.1001/jamadermatol.2023.5679","tag_type":"warning","value":"Seven of 90 patients were lost to follow-up, and those lost were predominantly male (6/1), which may bias results.","value_numeric":null}],"summaries":{"what_studied":"A median 7-year follow-up of the Ritux 3 trial compared first-line rituximab plus prednisone vs prednisone alone in pemphigus, with 5- and 7-year disease-free survival (DFS) without corticosteroids as the primary outcome. Of 90 randomized, 83 were evaluated at end of follow-up (median 87.3 months).","key_result":"DFS without steroids favored rituximab (5-year 76.7%, 7-year 72.1%) over prednisone alone (35.3% at both; P<.001); complete steroid-free remission occurred at any time in 43/44 (93%) vs 17/39 (39%). Relapses were less frequent (42.2% vs 83.7%; P<.001) and SAEs fewer (31 vs 58; 0.67 vs 1.32 per patient; P=.003); anti-Dsg1 ≥20 IU/mL and/or anti-Dsg3 ≥48 IU/mL predicted long-term relapse (PPV 0.83; NPV 0.94), and second-line rituximab had shorter DFS than first-line (P=.007).","limitations":"This is a secondary, long-term extension after initial randomization; some patients received rituximab as second-line. Seven of 90 were lost to follow-up, with a sex imbalance among those lost (male/female 6/1; P=0.04).","practice_takeaway":"For pemphigus, first-line rituximab (Ritux 3 regimen) achieved durable steroid-free control over 7 years with fewer relapses and SAEs than prednisone alone, without maintenance rituximab. Anti-Dsg1/3 thresholds (≥20/≥48 IU/mL) can help identify patients at higher risk of long-term relapse."}}],"initialOffset":2,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"Pemphigus"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"Pemphigus"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"Pemphigus"}}}]]

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