2363 Symptomatic Hepatic Sarcoidosis in a Hispanic Patient With Severely Elevated Liver Biochemical Tests

Abstract

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease of unknown etiology that typically predominates as a respiratory disease process. Hepatic involvement is uncommon and reported in only 11.5% of cases and even rarer is symptomatic hepatic involvement that occurs in approximately 5 to 30% of those cases. Epidemiological studies show substantial variations in extrapulmonary involvement based on race, with few studies on the Hispanic population. This case report presents a Hispanic female who underwent extensive workup and was diagnosed with symptomatic hepatic sarcoidosis. CASE DESCRIPTION/METHODS: A 27-year-old Hispanic woman with history of a mediastinal mass presented to the emergency department with an abrupt onset of right upper quadrant (RUQ) abdominal pain and nausea. Liver biochemical tests were severely elevated in a hepatocellular pattern. A RUQ ultrasound showed a dilated common bile duct (CBD) with a questionable stone in the CBD. Next, a magnetic resonance cholangiopancreatography was performed and demonstrated a normal CBD without biliary ductal dilatation or choledocholithiasis. Workup for chronic liver disease was negative, as well as extensive workup for other causes (Table 1). Then a liver biopsy was performed with histology demonstrating scattered non-necrotizing granulomas (Figure 1) with negative stains for mycobacterial and fungal organisms. Review of past medical records revealed a biopsy of her mediastinal mass with histology showing a non-necrotizing granulomas (Figure 2). A diagnosis of sarcoidosis was established and she was started on oral prednisone and referred to pulmonology. DISCUSSION: A diagnosis of sarcoidosis involves a suggestive history, granulomas in two organs, negative staining for acid fast bacilli, and a lack of drug-induced disease. The majority of patients with hepatic granulomas have primary biliary cholangitis (PBC). Screening for anti-mitochondrial antibodies and elevated IgM levels is important as these are present in >90% of those with PBC. Infectious diseases should be ruled out including tuberculosis, AIDS-related diseases, Q fever, and Brucellosis. Race is the most important risk factor for developing extrapulmonary sarcoidosis, possibly due to involvement of the reticuloendothelial system. Hispanics account for 18.1% of the U.S population. It is possible that Hispanics are at increased risk for extrapulmonary sarcoidosis compared to whites, but more Hispanic epidemiology studies are warranted.