Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature

Nikolic, Sara; Panić, Nikola; Hintikka, Elina Sofia; Dani, Lara; Rutkowski, Wiktor; Hedström, Aleksandra; Steiner, Corinna; Löhr, Matthias; Vujasinović, Miroslav

doi:10.1080/00365521.2021.1963837

Cited by 12 publications

(9 citation statements)

References 32 publications

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“…None of them relapsed during the 17-month follow-up period. These results confirm that RTX is effective in the treatment of AIP-1 by inducing remission and preventing relapse [ 83 ]. In some special cases, rituximab can be continued as maintenance therapy.…”

Section: Therapysupporting

confidence: 76%

Autoimmune Pancreatitis: From Pathogenesis to Treatment

Nista

Lucia

Manilla

et al. 2022

IJMS

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Autoimmune pancreatitis (AIP) is a rare disease. The diagnosis of AIP is difficult and should be made by a comprehensive evaluation of clinical, radiological, serological, and pathological findings. Two different types of AIP have been identified: autoimmune pancreatitis type 1 (AIP-1), which is considered a pancreatic manifestation of multiorgan disease related to IgG4, and autoimmune pancreatitis type 2 (AIP-2), which is considered a pancreas-specific disease not related to IgG4. Although the pathophysiological conditions seem to differ between type 1 and type 2 pancreatitis, both respond well to steroid medications. In this review, we focused on the pathogenesis of the disease to develop a tool that could facilitate diagnosis and lead to the discovery of new therapeutic strategies to combat autoimmune pancreatitis and its relapses. The standard therapy for AIP is oral administration of corticosteroids. Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. In selected patients, immunomodulators such as azathioprine are used to maintain remission. The strength of this review, compared with previous studies, is that it focuses on the clear difference between the two types of autoimmune pancreatitis with a clearly delineated and separate pathogenesis. In addition, the review also considers various therapeutic options, including biologic drugs, such as anti-tumor necrosis factor (TNF) therapy, a well-tolerated and effective second-line therapy for AIP type 2 relapses or steroid dependence. Other biologic therapies are also being explored that could provide a useful therapeutic alternative to corticosteroids and immunosuppressants, which are poorly tolerated due to significant side effects.

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Section: Therapysupporting

confidence: 76%

Autoimmune Pancreatitis: From Pathogenesis to Treatment

Nista

Lucia

Manilla

et al. 2022

IJMS

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“…Another retrospective study included 12 patients with AIP, who were treated with RTX for induction treatment (1000 mg biweekly in the first month) as well as for maintenance treatment (1000 mg repeated every 6 months). In this study, except for one patient who discontinued RTX for active tuberculosis, the other 11 patients sustained remission successfully during a median follow-up of 17 months 23. A case series study in six patients with IgG4-RD showed that methotrexate could sustain GC-free remission for 24 months (5/6 of patients) 24.…”

Section: Discussionmentioning

confidence: 63%

Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG4-RD (WInS IgG4-RD): an investigator-initiated, multicentre, open-label, randomised controlled trial

Peng,

Nie,

Zhou

et al. 2024

Ann Rheum Dis

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ObjectivesIgG4-related disease (IgG4-RD) is an immune-mediated, fibroinflammatory disease. Induction treatment with glucocorticoid (GC) is usually effective, but its tendency of relapse makes the strategy for maintenance treatment a challenge. The WInS IgG4-RD (withdraw immunosuppressants (IMs) and steroid in stable IgG4-RD) trial tested whether discontinuation of GC and IM was feasible in stable IgG4-RD.MethodsThe WInS IgG4-RD trial was a multicentre, open-label, randomised controlled trial. Patients with IgG4-RD receiving GC+IM as maintenance treatment with clinically quiescent disease for at least 12 months were randomised (1:1:1) into three groups: group 1: withdraw GC+IM; group 2: withdraw GC but maintain IM; group 3: maintain GC+IM. The primary outcome was the relapse rate of disease within 18 months. The secondary outcomes included the changes of IgG4-RD Responder Index (RI), Physician’s Global Assessment (PGA), serum IgG4 and IgG, as well as adverse events.ResultsOne hundred and forty-six patients were randomised, with 48 patients in group 1, 49 patients in group 2 and group 3, respectively. Within the 18-month follow-up period, disease relapse occurred in 25 out of 48 (52.1%) patients in group 1 vs 7 out of 49 (14.2%) in group 2 and 6 out of 49 (12.2%) in group 3 (p<0.001). The changes in RI and PGA were significantly higher in group 1 than in group 2 (p<0.001) or group 3 (p<0.001).ConclusionsThe maintenance of IMs, with or without low-dose GC, was found to be superior to withdraw GC+IM in preventing relapse for long-time stable IgG4-RD.Trial registration numberNCT04124861.

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“…Only a few cases have reported the coexistence of TB and AIP as well as the potential mechanism, most of whom had type 1 AIP or IgG4-related diseases. [38][39][40] A study identified crosstalk between T-helper 17 cells and neutrophils mediated through interleukin (IL)-17A as a potential mechanism for the recruitment and subsequent destruction of neutrophils into ductal and glandular blast cells, which may be the pathognomonic for type 2 AIP. 41 TB also causes an imbalance of T-and B-lymphocytes and up to 96.7% of differentially expressed genes in TB overlap with autoimmune diseases.…”

Section: Discussionmentioning

confidence: 99%

“…All TB infections in our study were detected before the diagnosis of IBD, which excluded opportunistic infections due to medical treatment. Only a few cases have reported the coexistence of TB and AIP as well as the potential mechanism, most of whom had type 1 AIP or IgG4‐related diseases 38–40 . A study identified crosstalk between T‐helper 17 cells and neutrophils mediated through interleukin (IL)‐17A as a potential mechanism for the recruitment and subsequent destruction of neutrophils into ductal and glandular blast cells, which may be the pathognomonic for type 2 AIP 41 .…”

Section: Discussionmentioning

confidence: 99%

Autoimmune pancreatitis associated with inflammatory bowel diseases: A retrospectively bidirectional case–control study in China

Bai

Ruan

et al. 2023

J of Digest Diseases

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AimAutoimmune pancreatitis (AIP) is a rare and enigmatic immune‐mediated inflammatory diseases associated with inflammatory bowel disease (IBD). We aimed to describe the prevalence, characteristics, and associated factors of AIP‐IBD patients in China.MethodsWe performed a retrospective bidirectional case–control study from 1998 to 2021. The diagnosis of IBD was based on the European Crohn's and Colitis Organisation guidelines and of AIP was based on the International Consensus Diagnostic Criteria. IBD controls were matched by age (±1), sex, and IBD type at a ratio of 1:4, while AIP controls were matched by the frequency of AIP types.ResultsThe age‐standardized prevalence of AIP‐IBD patients in the IBD and AIP population were 292.04 and 8151.93 per 100 000, respectively. IBD patients had a higher risk of suffering AIP compared to non‐IBD patients (OR: 8.4, CI: 4.7–14.9, p < 0.0001), as well as AIP patients developing IBD compared to general population in China. The mean age at diagnosis of IBD was 34.8, and of AIP was 50. Seven cases were first diagnosed with IBD, and the average IBD duration was 94.8 months, while 26.1 months for AIP. Independent factors associated with AIP was tuberculosis infection (p < 0.0001). The occult blood test (p = 0.008) was independently associated with IBD.ConclusionsWe found most AIP‐IBD patients had UC or type 2 AIP. The IBD population is more likely to develop AIP compared to the general population, vice versa. Tuberculosis infection were associated with AIP, and occult blood test was associated with IBD.This article is protected by copyright. All rights reserved.

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Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature

Cited by 12 publications

References 32 publications

Autoimmune Pancreatitis: From Pathogenesis to Treatment

Autoimmune Pancreatitis: From Pathogenesis to Treatment

Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG4-RD (WInS IgG4-RD): an investigator-initiated, multicentre, open-label, randomised controlled trial

Autoimmune pancreatitis associated with inflammatory bowel diseases: A retrospectively bidirectional case–control study in China

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