Faculty Opinions recommendation of Autoimmune pancreatitis (AIP) type 1 and type 2: an international consensus study on histopathologic diagnostic criteria.

Autoimmune pancreatitis (AIP) is a subcategory of chronic pancreatitis that is highly responsive to steroids. The term was first proposed in 1995 by Yoshida and colleagues, and since its discovery, the diagnosis of AIP has dramatically increased. AIP is a chronic fibroinflammatory disease characterized by lymphoplasmacytic infiltrates and fibrosis on histology. There are two distinct subtypes: type 1 AIP is the pancreatic manifestation of a systemic serum immunoglobulin G subtype 4–related disease (IgG4-RD) and type 2 AIP is described clinically as idiopathic duct-centric pancreatitis and has no association with IgG4. Clinically, AIP presents most commonly as obstructive jaundice in type 1 AIP and can present as acute pancreatitis in type 2 AIP. The diagnostic criteria include histology, imaging findings, and responsiveness to steroids as well as laboratory findings and other organ involvement. The mainstay of treatment is steroid therapy, with immunomodulators such as rituximab used for maintenance or relapsing disease. Long-term complications of AIP include pancreatic insufficiency and are often associated with relapsing disease. This review contains 45 references, 1 figure, and 2 tables. Key Words: autoimmune pancreatitis, chronic pancreatitis, EUS-guided biopsy, IgG4, immunomodulatory, obstructive jaundice, pancreas mass, steroid

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Surgery in autoimmune pancreatitis

Digestive Surgery ◽

10.1159/000521490 ◽

2021 ◽

Author(s):

Sara Nikolic ◽

Poya Ghorbani ◽

Raffaella Pozzi Mucelli ◽

Sam Ghazi ◽

Francisco Baldaque- Silva ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

University Hospital ◽

Premalignant Lesions ◽

Single Centre ◽

Age At Surgery ◽

Diagnostic Work ◽

Work Up

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.

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International Consensus Diagnostic Criteria for autoimmune pancreatitis -Guidelines of the International Association of Pancreatology-

Suizo ◽

10.2958/suizo.26.684 ◽

2011 ◽

Vol 26 (6) ◽

pp. 684-698 ◽

Cited By ~ 2

Author(s):

Tooru SHIMOSEGAWA ◽

Kazuichi OKAZAKI ◽

Terumi KAMISAWA ◽

Shigeyuki KAWA ◽

Kenji NOTOHARA

Keyword(s):

Diagnostic Criteria ◽

Autoimmune Pancreatitis ◽

International Association ◽

International Consensus

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Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis

Journal of Clinical Medicine ◽

10.3390/jcm8020258 ◽

2019 ◽

Vol 8 (2) ◽

pp. 258

Author(s):

Miroslav Vujasinovic ◽

Raffaella Pozzi Mucelli ◽

Roberto Valente ◽

Caroline Verbeke ◽

Stephan Haas ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

Lymphocytic Infiltration ◽

Normal Serum ◽

International Consensus ◽

Kidney Involvement ◽

Type 1 Autoimmune Pancreatitis ◽

Elevated Creatinine ◽

Imaging Signs

Introduction: Autoimmune pancreatitis (AIP) type 1 is a special form of chronic pancreatitis with a strong lymphocytic infiltration as the pathological hallmark and other organ involvement (OOI). IgG4-related kidney disease (IgG4-RKD) was first reported as an extrapancreatic manifestation of AIP in 2004. The aim of the present study was to determine the frequency and clinical impact of kidney lesions observed in patients with AIP type 1. Methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients with a histologically proven or highly probable diagnosis of AIP according to the International Consensus Diagnostic Criteria (ICDC) classification. Results: Seventy-one patients with AIP were evaluated. AIP type 1 was diagnosed in 62 (87%) patients. Kidney involvement was present in 17 (27.4%) patients with AIP type 1: 15 (88.2%) males and 2 (11.8%) females. Laboratory and/or imaging signs of kidney involvement were presented at the time of AIP diagnosis in eight (47.1%) patients. In other patients, the onset of kidney involvement occurred between four months and eight years following diagnosis. At the time of the diagnosis of kidney involvement, eight (47.1%) patients showed elevated creatinine, and nine (52.9%) patients showed normal serum creatinine. None of the patients were treated with dialysis. Conclusions: IgG4-RKD was present in 27.4% of patients with AIP type 1, with male gender predominance. In cases of early diagnosis and cortisone treatment, the clinical course was mild in most cases. Regular laboratory control of renal function should be a part of the follow-up of patients with AIP type 1.

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Autoimmune Pancreatitis - A Riddle Wrapped in an Enigma

Digestive Diseases ◽

10.1159/000445234 ◽

2016 ◽

Vol 34 (5) ◽

pp. 532-539 ◽

Cited By ~ 3

Author(s):

George J. Webster

Keyword(s):

Autoimmune Pancreatitis ◽

Clinical Laboratory ◽

Clinical Manifestations ◽

Organ Involvement ◽

Clinical Relapse ◽

Biliary Disease ◽

Disease Course ◽

International Consensus ◽

Igg4 Related Disease

Autoimmune pancreatitis (AIP) was recognized as a clinical entity, at least in the West little more than 10 years ago. Since then, studies globally, and international collaboration, have led to important advances in our understanding of its clinical features, disease course, and management, although the aetiopathogenesis of this curious disease remains to be fully elucidated. Types 1 and 2 AIP have been described, of which type 1 is the commonest form, and best defined. International consensus now recognizes it as one of the many clinical manifestations of IgG4-related disease, and is now termed IgG4-related pancreatitis (IgG4-RP). The disease is not confined to a particular race, gender, or age, but often presents after the fifth decade in men. A common presentation is with jaundice due to low bile duct obstruction related to diffuse pancreatic enlargement (historically often leading to a misdiagnosis of cancer). Acute pancreatitis is unusual. Other organ involvement is a particular feature, including biliary disease, retroperitoneal fibrosis, generalized lymphadenopathy, renal, and lung involvement. No single test makes the diagnosis, and diagnostic criteria for type 1 AIP/IgG4-RP, which incorporate clinical, laboratory, radiological, pathological, and therapeutic parameters should be applied. A particular attempt should be made to make a histological diagnosis, which is characterized by an IgG4-positive lymphoplasmacytic infiltrate. Management is not based on randomized studies, but corticosteroids are the mainstay of treatment, providing rapid clinical and radiological benefit. However, clinical relapse is common (particularly in type 1 AIP, and in those with associated other organ involvement). Additional immunosuppression may be required, including azathioprine, and rituximab may play an emerging role. The disease course is variable, but loss of organ function (especially pancreatic exocrine failure and pancreatic atrophy) may occur.

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