ArticleJournal of clinical medicine2021
A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab.
Article in Journal of clinical medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 30 citations in OpenAlex.
- Management of IgG4-Related Disease.Current rheumatology reports · 2026Review
- Bridging the Gap: Guideline Adherence in IgG4-Related Digestive Disease.United European gastroenterology journal · 2025Article
- New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach.Journal of clinical medicine · 2025Review
- Clinicopathological Pearls and Diagnostic Pitfalls in IgG4-Related Disease: Challenging Case Series and Literature Review.Diagnostics (Basel, Switzerland) · 2025Article
- A protocol for targeted B-lymphocyte depletion for the treatment of IgG4-related disease.Rheumatology (Oxford, England) · 2025Article
- Treatment approaches in autoimmune pancreatitis (Review).Biomedical reports · 2024Review
- Renal Manifestations of IgG4-Related Disease: A Concise Review.International journal of nephrology · 2024Review
- The spectrum of B cells in the pathogenesis, diagnosis and therapeutic applications of immunoglobulin G4-related disease.Clinical & translational immunology · 2023Review
- Autoimmune Pancreatitis: From Pathogenesis to Treatment.International journal of molecular sciences · 2022Review
- Updates on the Treatment of Pancreatic Diseases: Focus on Surgery, Electrochemotherapy and Rituximab.Journal of clinical medicine · 2022Article
- Autoimmune pancreatitis: What we know so far.JGH open : an open access journal of gastroenterology and hepatology · 2022Review
- Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency.Case reports in immunology · 2021Article
- IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.Canadian journal of gastroenterology & hepatology · 2021Review
- Case Report: IgG4-related disease presenting with prominent oculomotor nerve palsy.Frontiers in neurologyArticle
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesIgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity.
methodsWe retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019.
resultsPatients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX.
conclusionRTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6-9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.