A Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD)

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

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IgG4-related disease responder index but not serum IgG4 correlates with disease activity and the risk of 1-year relapse in type 1 autoimmune pancreatitis

Pancreatology ◽

10.1016/j.pan.2019.05.367 ◽

2019 ◽

Vol 19 ◽

pp. S135

Author(s):

Filippo Vieceli ◽

Giulia De Marchi ◽

Antonio Amodio ◽

Lorenzo Brozzi ◽

Pietro Campagnola ◽

...

Keyword(s):

Disease Activity ◽

Autoimmune Pancreatitis ◽

Related Disease ◽

Igg4 Related Disease ◽

Serum Igg4 ◽

Type 1 Autoimmune Pancreatitis

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Immunoglobulin G4-Related Disease Responder Index Correlates With the Risk of 1-Year Relapse in Type 1 Autoimmune Pancreatitis

Pancreas ◽

10.1097/mpa.0000000000001843 ◽

2021 ◽

Vol Publish Ahead of Print ◽

Author(s):

Teresa Marzia Rogger ◽

Giulia De Marchi ◽

Lorenzo Brozzi ◽

Antonio Amodio ◽

Giovanni Orsolini ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

Immunoglobulin G4 ◽

Related Disease ◽

Type 1 Autoimmune Pancreatitis

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IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

Case Reports in Gastrointestinal Medicine ◽

10.1155/2015/591360 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6

Author(s):

Allon Kahn ◽

Anitha D. Yadav ◽

M. Edwyn Harrison

Keyword(s):

Autoimmune Pancreatitis ◽

Sclerosing Cholangitis ◽

Clinical Presentation ◽

Surgical Intervention ◽

Comprehensive Evaluation ◽

Elevated Serum ◽

Igg4 Related Disease ◽

Serum Igg4 ◽

Type 1 Autoimmune Pancreatitis

IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

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A Patient with Autoimmune Pancreatitis Type 1 with Previously Known Lymphadenopathy, Both in the Context of IgG4-related Disease

American Journal of Case Reports ◽

10.12659/ajcr.895059 ◽

2015 ◽

Vol 16 ◽

pp. 790-793

Author(s):

Fazil M. Alidjan ◽

Faiz Karim ◽

Rob M. Verdijk ◽

Joost W. van Esser ◽

Marianne J. van Heerde

Keyword(s):

Autoimmune Pancreatitis ◽

Related Disease ◽

Igg4 Related Disease

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A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

Journal of Clinical Medicine ◽

10.3390/jcm10061329 ◽

2021 ◽

Vol 10 (6) ◽

pp. 1329

Author(s):

Johanna Backhus ◽

Christian Neumann ◽

Lukas Perkhofer ◽

Lucas A Schulte ◽

Benjamin Mayer ◽

...

Keyword(s):

Disease Activity ◽

Clinical Activity ◽

Inflammatory Disorder ◽

International Consensus ◽

Steroid Pulse ◽

Related Disease ◽

Igg4 Related Disease

Objectives: IgG4-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. Methods: We 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. Results: Patients 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. Conclusion: RTX 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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