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6 May 2015 - Case of the Week #351

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Case of the Week #351

Clinical History:
A 55 year old man had a common hepatic duct stricture, which was biopsied. Subsequently, he underwent a cholecystectomy and partial biliary tree resection, with sections below.

Micro images:


What is your diagnosis?































Diagnosis:
IgG4-related sclerosing cholangitis

Discussion:
An IgG4 immunostain from the outside facility (image not available) showed > 100 IgG4+ plasma cells per HPF.

Primary sclerosing cholangitis occurs predominantly in men, usually under age 45 years. It often has an autoimmune origin, as 50-70% also have inflammatory bowel disease (particularly ulcerative colitis), although only 4% with ulcerative colitis have primary sclerosing cholangitis. Histologic features include:

  • Fibrosing cholangitis of intra- and extrahepatic bile ducts (large and small) with lymphocytic infiltration
  • Progressive atrophy of bile duct epithelium and obliteration of the lumen, with diffuse bile ductular proliferation
  • "Onion skin" fibrosis around affected ducts, which later disappears, leaving a cord-like fibrous scar
  • The remaining ducts are ectatic and inflamed, and often elongated, with variable portal eosinophils
  • Cirrhotic nodules, when present, have a jigsaw pattern due to portal to portal fibrosis; also present are gallbladder sclerosis and prominent lymphoid aggregates
  • Post-transplant recurrences exhibit bile duct strictures and nonspecific autoimmune hepatitis with variable fibrosis (Eur Radiol 2014;24:1039)
A newly recognized cause of secondary sclerosing cholangitis is IgG4 associated cholangitis. It is typically associated with autoimmune pancreatitis, although isolated intrapancreatic cases without autoimmune pancreatitis have been reported (World J Gastroenterol 2015;21:1334).

Patients with tissue IgG4 positivity have a more aggressive clinical course, including shorter time to transplant, and higher likelihood of recurrence (Am J Surg Pathol 2010;34:88). Diagnostic criteria includes 10+ IgG4+ plasma cells/HPF at biopsy, although a cutoff of 50 has been recommended (Scand J Gastroenterol 2015;50:447).

Steroid treatment is standard. Assessment of steroid responsiveness, a diagnostic criteria, is recommended after initiation of treatment at 5 and 10 days, by testing serum total bilirubin, alanine aminotransferase and alkaline phosphatase, and at 1-2 weeks by endoscopic retrograde cholangiography and magnetic resonance cholangiopancreatography (Adv Med Sci 2015;60:211).

The differential diagnosis includes cholangiocarcinoma / bile duct carcinoma, because the entities may be clinically similar (Int Surg 2015;100:480, Hepatogastroenterology 2014;61:1852, Hum Pathol 2014;45:1722).

Nat Pernick, M.D., President
and Shivani Thakore, Associate Medical Editor
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