SARCOIDOSIS—HEPATIC INVOLVEMENT: PRESENTATION OF A CASE WITH FATAL LIVER INVOLVEMENT, INCLUDING AUTOPSY FINDINGS AND REVIEW OF THE EVIDENCE FOR SARCOID INVOLVEMENT OF THE LIVER AS FOUND IN THE LITERATURE

Abstract

A case of sarcoidosis with fatal liver involvement diagnosed ante mortem by skin biopsy and showing roentgenographic intrathoracic lymph node involvement, skin lesions, transient ecg. changes, ascites, jaundice, and other evidence of marked liver function impairment is presented. Autopsy revealed characteristic microscopic tubercles of sarcoidosis in the lymph nodes, lungs, liver, spleen, and skin. In addition, the liver showed marked biliary stasis, bile duct proliferation, and hyalinized connective tissue encroaching upon and altering the normal hepatic architecture. Review of the literature for clinicopathologic features reveals 79.5% of 571 patients to be in Negroes and in another study 88% of 226 patients came from the southern U.S., and souther-born Negroes outnumbered the northern-born Negroes 15.5 to 1. Of the 571 cases, 59% were in females, and the average age at the time of diagnosis was 30.25 yrs., while the average age at the time of death (138 autopsied cases) was 34.2 yrs. Clinical evidence for liver involvement (excluding hepatomegaly) was found in only 7% of 710 but in 318 cases with adequate serum protein studies 61% showed a globulin fraction exceeding 2.75 g./100 cc., indicating some alteration in liver function. In 63 cases with a previously established diagnosis of sarcoidosis, liver biopsy was positive in 76.25%. Analysis of 138 autopsy protocols reveals the liver contained the typical granulomatous nodules in 66.5% (after elimination of 21 cases because of inadequate data, microscopic evidence, or failure to exclude other diseases simulating sarcoidosis). Only 3 previous cases of jaundice were found to be due to specific hepatic sarcoid involvement, and in none of these was the liver involvement the primary cause of death. Sarcoidosis as a cause of liver disease is not common, being found in only 2% of 1063 liver biopsies done on patients with liver disease from all causes. Although approx. 15% of all sarcoidosis cases are believed to be associated with tuberculosis during some phase of the disease (90% show negative tuberculin tests), no definite relationship between the 2 diseases has been established and the etiology remains in doubt. There is no specific treatment at this time, although recent reports indicate some promise for the steroid hormones.