Autoimmune pancreatitis in a patient presenting with obstructive jaundice and pancreatic mass

Autoimmune pancreatitis (AIP) is a rare entity that is extremely uncommon in children. Its diagnosis is also a clinical challenge. This form of chronic pancreatitis often presents itself with obstructive jaundice and/or a pancreatic mass and it is sometimes misdiagnosed as pancreatic cancer. We describe the case of a 13-year-old boy with obstructive jaundice and a 4 cm mass in the head of the pancreas that was diagnosed as AIP with associated ulcerative colitis.

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Autoimmune pancreatitis: an important diagnostic consideration in obstructive jaundice due to a pancreatic mass lesion

Internal Medicine Journal ◽

10.1111/j.1445-5994.2010.02300.x ◽

2010 ◽

Vol 40 (10) ◽

pp. 720-725 ◽

Cited By ~ 1

Author(s):

M. P. Swan ◽

M. J. Bourke ◽

A. D. Hopper ◽

V. Kwan ◽

S. J. Williams

Keyword(s):

Obstructive Jaundice ◽

Autoimmune Pancreatitis ◽

Mass Lesion ◽

Pancreatic Mass

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P045 Autoimmune pancreatitis in a child presenting as a pancreatic mass

Digestive and Liver Disease ◽

10.1016/s1590-8658(18)31044-2 ◽

2018 ◽

Vol 50 (4) ◽

pp. e375

Author(s):

M. Distefano ◽

A. Perduca ◽

L. Parola ◽

P. Sgaramella ◽

G. Barera

Keyword(s):

Autoimmune Pancreatitis ◽

Pancreatic Mass

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Autoimmune Pancreatitis

10.2310/gastro.5467 ◽

2018 ◽

Author(s):

Allison L Yang ◽

Julia McNabb-Baltar

Keyword(s):

Chronic Pancreatitis ◽

Obstructive Jaundice ◽

Autoimmune Pancreatitis ◽

Pancreatic Insufficiency ◽

Imaging Findings ◽

Laboratory Findings ◽

Related Disease

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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Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting

Pancreatology ◽

10.1016/j.pan.2016.03.017 ◽

2016 ◽

Vol 16 (3) ◽

pp. 391-396 ◽

Cited By ~ 19

Author(s):

Yan Bi ◽

Phil A. Hart ◽

Ryan Law ◽

Jonathan E. Clain ◽

Michael B. Farnell ◽

...

Keyword(s):

Obstructive Jaundice ◽

Autoimmune Pancreatitis ◽

Biliary Stenting

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Validity and safety of corticosteroids alone without biliary stenting for obstructive jaundice in autoimmune pancreatitis

Pancreatology ◽

10.1016/j.pan.2020.10.034 ◽

2020 ◽

Vol 20 (8) ◽

pp. 1793-1795

Author(s):

Yunlu Feng ◽

Shengyu Zhang ◽

Tao Guo ◽

Weiyang Zheng ◽

Dong Wu ◽

...

Keyword(s):

Obstructive Jaundice ◽

Autoimmune Pancreatitis ◽

Biliary Stenting

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Deciphering Autoimmune Pancreatitis, a Great Mimicker: Case Report and Review of the Literature

Case Reports in Gastrointestinal Medicine ◽

10.1155/2015/924532 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6

Author(s):

Satya Allaparthi ◽

Mohammed Sageer ◽

Mark J. Sterling

Keyword(s):

Obstructive Jaundice ◽

Autoimmune Pancreatitis ◽

Epigastric Pain ◽

Hepatic Duct ◽

Liver Function Tests ◽

Pancreatic Disease ◽

Common Hepatic Duct ◽

Caucasian Woman ◽

Distal Common Bile Duct ◽

Biliary Sphincterotomy

Background. Autoimmune pancreatitis (AIP) is an atypical chronic inflammatory pancreatic disease that appears to involve autoimmune mechanisms. In recent years, AIP has presented as a new clinical entity with its protean pancreaticobiliary and systemic presentations. Its unique pathology and overlap of clinical and radiological features and absence of serological markers foster the disease’s unique position. We report a case of diffuse type 1 autoimmune pancreatitis with obstructive jaundice managed with biliary sphincterotomy, stent placement, and corticosteroids. A 50-year-old Caucasian woman presented to our hospital with epigastric pain, nausea, vomiting, and jaundice. Workup showed elevated liver function tests (LFT) suggestive of obstructive jaundice, MRCP done showed diffusely enlarged abnormal appearing pancreas with loss of normal lobulated contours, and IgG4 antibody level was 765 mg/dL. EUS revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD). ERCP showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy, and placement of stent that led to significant improvement in the symptoms and bilirubin level. Based on clinical, radiological, and immunological findings, a definitive diagnosis of AIP was made. Patient was started on prednisone 40 mg/day and she clinically responded in 4 weeks.

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Serum Immunoglobulin G Fraction 4 Levels in Pancreatic Cancer: Elevations Not Associated With Autoimmune Pancreatitis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2008-132-48-sigfli ◽

2008 ◽

Vol 132 (1) ◽

pp. 48-53 ◽

Cited By ~ 8

Author(s):

Amit Raina ◽

Alyssa M. Krasinskas ◽

Julia B. Greer ◽

Janette Lamb ◽

Erin Fink ◽

...

Keyword(s):

Pancreatic Cancer ◽

Cancer Patients ◽

Immunoglobulin G ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Immunoglobulin ◽

Pancreatic Mass ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Abstract Context.—Autoimmune pancreatitis is an uncommon, inflammatory disease of the pancreas that presents with clinical features, such as painless jaundice and a pancreatic mass, similar to those caused by pancreatic cancer. Patients with autoimmune pancreatitis frequently have elevated serum immunoglobulin G fraction 4 (IgG4) levels, and their pancreatic tissue may show IgG4-positive plasma cell infiltration. It is imperative to differentiate autoimmune pancreatitis from pancreatic cancer because autoimmune pancreatitis typically responds to corticosteroid treatment. A previous Japanese study reported that serum IgG4 greater than 135 mg/dL was 97% specific and 95% sensitive in predicting autoimmune pancreatitis. Objective.—To prospectively measure serum IgG4 levels in pancreatic cancer patients to ascertain whether increased levels might be present in this North American population. Design.—We collected blood samples and phenotypic information on 71 consecutive pancreatic cancer patients and 103 healthy controls who visited our clinics between October 2004 and April 2006. IgG4 levels were determined using a single radial immunodiffusion assay. A serum IgG4 level greater than 135 mg/dL was considered elevated. Results.—Five cancer patients had IgG4 elevation, with a mean serum IgG4 level of 160.8 mg/dL. None of our cancer patients with plasma IgG4 elevation demonstrated evidence of autoimmune pancreatitis. One control subject demonstrated elevated serum IgG4 unrelated to identified etiology. Conclusions.—As many as 7% of patients with pancreatic cancer have serum IgG4 levels above 135 mg/dL. In patients with pancreatic mass lesions and suspicion of cancer, an IgG4 level measuring between 135 and 200 mg/dL should be interpreted cautiously and not accepted as diagnostic of autoimmune pancreatitis without further evaluation.

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