Answer
From question on page 1346
The CT scan shows intra‐hepatic and extra‐hepatic ductal dilatation with extensive extrahepatic lymphadenopathy. The histology of the lymph‐node biopsy shows non‐caseating histiocytic granulomata with giant cells and asteroid bodies. The histology shows that stains for acid‐fast bacilli and fungi were negative.
Serum angiotensinogen‐converting enzyme was raised, at 61 U/l (normal range 25–82). A diagnosis of sarcoidosis causing obstructive jaundice was made, and the patient was started on corticosteroids. Liver‐function tests returned to within normal limits and the patient's symptoms completely resolved.
A repeat CT scan of the abdomen, 11 months after the initial consultation, showed complete resolution of intrahepatic bile duct dilatation and a marked improvement in lymphadenopathy.
Sarcoidosis is a systemic inflammatory disease characterised by the formation of non‐caseating granulomas. The aetiology remains unknown, but speculation has focused on an unidentified environmental factor triggering a response in susceptible individuals. The prevalence is highest in the USA, Sweden, Denmark and Japan, and lowest in Spain, China and South America.1
Sarcoidosis can affect most systems, but pulmonary presentations are the most common. The abdominal viscera are often involved, but do usually produce symptoms.2 There are very few reports in the literature of sarcoidosis causing cholestasis. Intrahepatic involvement of the biliary tree through granuloma affecting the portal triads can produce a cholestatic picture, and may mimic primary biliary cirrhosis, although there are no antimitochondrial antibodies.3 Involvement of the extrahepatic ducts can produce stricturing resembling cholangiocarcinoma.4 Enlarged portal nodes can compress the biliary tree to cause obstructive jaundice.5 The disease can present with severe cholestasis resembling primary sclerosing cholangitis6 Patients with this condition usually respond well to steroid therapy.
References
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