Skip Navigation LinksHome > April 2012 - Volume 54 - Issue 4 > Autoimmune Cholecystitis
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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e31823a08a6
Image of the Month

Autoimmune Cholecystitis

Memon, Zebunnissa; Moya, Diana A.; Baker, Robert; Kozielski, Rafal; Baker, Susan

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Author Information

Digestive Disease and Nutrition Center, SUNY-Buffalo, Women and Children's Hospital of Buffalo, Buffalo, NY

Address correspondence and reprint requests to Zebunnissa Memon, MD, Digestive Disease and Nutrition Center, SUNY-Buffalo, Women and Children's Hospital of Buffalo, Buffalo, NY 14222 (e-mail: zmemon@upa.chob.edu).

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

The authors have no conflicts of interest.

A 16-year-old boy experienced chronic vague abdominal pain, dark urine, acholic stools, and jaundice. Laboratory studies were consistent with pancreatitis and hepatitis (elevated lipase, aspartate aminotransferase, alanine aminotransferase, γ-glutamyl transferase, direct bilirubin, positive anti-nuclear antibody, and anti-smooth muscle antibody). An endoscopic retrograde cholangiopancreatography was normal. Abdominal magnetic resonance imaging showed biliary duct dilatation with normal pancreas. He had elevated immunoglobulin (Ig) G of 4080 mg/dL. He underwent a cholecystectomy and a liver biopsy. The liver biopsy was consistent with autoimmune hepatitis. The gallbladder histology, pictured above, showed diffuse lymphoplasmacytic acalculous cholecystitis. There was portal inflammation with relative sparing of intrahepatic bile ducts and periductal inflammation but no overt cholangitis. No cholestasis or periductal fibrosis was seen. Immunohistochemistry staining for IgG4 was positive for plasma cells in liver and gallbladder specimens, diagnostic of autoimmune cholecystitis. He was treated with corticosteroids and 6-mercaptopurine. His transaminases, lipase, and amylase returned to normal.

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Autoimmune cholangitis is the biliary manifestation of a multisystem fibroinflammatory disorder. IgG4-associated autoimmune cholangiopathy may occur in the presence of autoimmune pancreatitis and hepatitis. Pathologically, there is a characteristic lymphoplasmacytic infiltrate and abundance in IgG4-positive cells. Although responsive to steroids, relapse is common after early withdrawal of treatment.

Copyright 2012 by ESPGHAN and NASPGHAN

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