HEPATIC SARCOIDOSIS - CLINICOPATHOLOGICAL FEATURES IN 100 PATIENTS

The patterns of hepatic injury were studied in 100 patients with a dia gnosis of sarcoidosis and clinical evidence of liver disease that led to diagnostic liver biopsy. Granulomas were present in all patients; t hey occupied from < 1% to > 90% of the total volume of tissue examined and were most often located in the portal/periportal region. In none of the 100 cases were infectious organisms identified by special stain s, culture, or serology. In 99% of cases, these granulomas were noncas eating; in one of the 100 cases central caseation was noted. In additi on to the granulomas present in all biopsies, three broad categories o f histologic change were found: cholestatic (58%), necroinflammatory ( 41%), and vascular (20%). Among those with cholestasis, 19 patients ha d bile duct lesions similar to primary biliary cirrhosis, whereas anot her 13 had a pattern of periductal fibrosis reminiscent of primary scl erosing cholangitis. In 37 patients with chronic cholestasis, a decrea se in the number of bile ducts (ductopenia) was noted. Twelve patients had an acute cholangitis suggestive of mechanical obstruction-althoug h no clinical evidence of ductal obstruction was found. Necroinflammat ory changes included spotty necrosis suggesting hepatitis of diverse e tiologies (including viral infection and drug reaction) and chronic po rtal inflammation suggestive of chronic active hepatitis. Vascular cha nges consisted of sinusoidal dilatation (14 cases) and nodular regener ative hyperplasia (9 cases). In 6% of the patients, the only changes i n the biopsy were those of granulomatous inflammation; each of these p atients had a dominant mass (''sarcoidoma''), which had been biopsied to rule out tumor. Fibrosis was seen in 21% of the biopsies-periportal (13%), bridging (2%), or cirrhosis (6%). It is clear that sarcoidosis can cause progressive liver disease with a wide array of histologic f eatures that can mimic those of other primary liver diseases.