Hepatic Granulomas: An Unusual Presentation of Sarcoidosis

Abstract

A 49-year-old previously healthy male presented with 5 weeks of fatigue, malaise, nonproductive cough, and headache and elevated liver associated enzymes in a hepatocellular fashion. He reported no high risk sexual behavior and rare alcohol use; he denied illicit drug use, blood transfusions, tattoos, or recent travel. He endorsed occasional NSAID use prior to presentation, but denied other OTC or herbal medicines. He denied fevers, chills, weight loss, change in appetite, pruritus, abdominal pain, or change in bowel habits. On examination there was no stigmata of liver disease. His chronic viral hepatitis panel was negative as well as the rest of his work up; α-1 antitrypsin and ferritin; ANA negative, AMA negative, but anti-smooth muscle antibody weakly positive. A liver biopsy revealed diffuse non-caseated granulomata throughout all zones of the hepatic parenchyma without evidence of fibrosis consistent with granulomatous hepatitis. An ACE level was drawn due to concern for extrapulmonary sarcoidosis, which was elevated (93 U/L). Chest radiograph revealed a 2cm spiculated density in the right mid-lung but failed to demonstrate the hallmark hilar lymphadenopathy and reticular opacities of pulmonary sarcoidosis. Subsequent CT chest revealed a segmental opacity in the right middle lobe, and mediastinal lymphadenopathy. Hepatic sarcoidosis must be differentiated from other causes of granulomatous hepatitis including drug reaction, infection, and PBC. The negative AMA and normal serum IgM make PBC unlikely. Fungal and mycobacterial stains failed to demonstrate any fungal or mycobacterial organisms. Quantiferon, CMV serology, EBV PCR, Histoplasma serology, Lyme disease, and HIV testing were all negative. The elevated ACE level and CT findings support the diagnosis of hepatic sarcoidosis. Over the next few months with treatment, the patients' symptoms resolved and liver enzymes began to downtrend.Figure 1Sarcoidosis is a disease of unclear etiology, characterized by non-caseating granulomas in involved organs. Only 10% of patients with systemic sarcoidosis develop abnormal liver enzymes. Usually liver involvement is clinically silent and symptomatic patients usually present with a cholestatic pattern of liver injury. Our patient presented with several weeks of fatigue and found to have liver injury with a hepatocellular pattern which is a rare presentation of granulomatous hepatitis most likely representing hepatic sarcoidosis.