Severe cholestatic granuloumatous hepatitis in sarcoidosis disease with minimal pulmonary involvement

Extrapulmonary manifestations of sarcoidosis are frequently reported. Liver involvement is usually inapparent, with only mild elevation of liver function tests, whereas progressive cholestasis and/or cirrhosis are rarely reported. Sarcoidosis prevalence differs among racial and ethnic groups, and it is known to affect blacks more acutely and more severely than it does people of other races. We report the case of a black nigerian 35-yr old woman, living in Italy since 1988, admitted because fever, itching and increased cholestatic liver tests (GT 1837 IU, ALP 3854 IU, total bilirubin 3.5 mg/dl, AST 71 IU, ALT 84 IU, Cholesterol 448 mg/dl). Abdominal US revealed gallstones and associated cholecystitis, and hepatosplenomegaly. She underwent laparoscopic cholecystectomy: during the procedure an enlarged lymph node close to the gallbladder was collected and a liver biopsy was performed. Histology showed multiple confluent non caseating granulomas, with epitheliod and multinucleated giant cells. All serological and antibodies tests, and microbiological cultures were negative. Chest X-ray, CT and HRCT were normal, whereas abdominal CT confirmed an important hepatosplenomegaly, with numerous hypodense nodular splenic lesions, and hepatic hilar-node enlargement. No significant pulmonary physiologic abnormalities were detected, nevertheless a broncoscopy was performed, with transbronchial lung biopsy and bronco-alveolar-lavage collections. Lung biopsies confirmed the presence of granulomatous involvement and the BAL showed a typical sarcoidosis pattern. Serum ACE (angiotensin-converting-enzyme) activity was 70 U/L. Corticosteroid therapy was started (prednisolone 50 mg daily, with gradual tapering of the dose), plus azatioprine (150 mg daily) and ursodeoxycholic acid (1350 mg daily). After six month of treatment transaminases, bilirubin and ACE levels were in the normal range, whereas GT and ALP decreased significantly (1249 and 1331, respectively). This single case of sarcoidosis presented to the clinician almost exclusively with liver disease; it confirms that in Africans the disease is characterized by more severe extrapulmonary involvement. The consideration of sarcoidosis in such cases is of utmost importance, since the differential diagnosis of hepatic granulomas includes infectious diseases in which treatment with corticosteroids could be fatal.