S2643 Hepatic Sarcoidosis Manifesting With Severe Hypercalcemia in an Elderly Woman: A Rare Presentation

Abstract

INTRODUCTION: Sarcoidosis is a chronic, multisystemic non-caseous granulomatous disease of unknown origin. Exclusive liver involvement without lung disease is less frequent, documented in only about 13% of patients with systemic sarcoidosis. The diagnosis of liver sarcoidosis is difficult, because symptoms or functional derangement due to involvement of the liver are uncommon in sarcoidosis. CASE DESCRIPTION/METHODS: An 83 year old woman presented with fever, chills and fatigue of 1 week duration. She further reported no alcohol or drug use, prior blood transfusions and use of herbal supplements. Her exam was unremarkable and initial laboratory data was significant for eosinophilia of 0.6 k/uL and the following liver tests: ALP 613 U/L, AST 234 U/L, ALT 224 U/L, total bilirubin 0.8 mg/dL, INR 1.0 and Gamma Glutamyl Transferase (GGT) 518 U/L. Infectious workup was negative for HBV, HCV, CMV, EBV and Quantiferon. Magnetic resonance imaging of the liver was negative for masses and biliary dilation. Chest CT was negative for pulmonary fibrosis and hilar adenopathy. Immunological workup came back negative. The patient’s ALP was of macrohepatic source and peaked to 1251 U/L over the following week, serum calcium rapidly increased to 13.6 mg/dl and Angiotensin-1 converting enzyme (ACE) was >80 U/L. At this point a liver biopsy was conducted which showed non-necrotic granulomata in a lobular and periportal distribution consistent with sarcoidosis of the liver. The patient was placed on oral prednisone after which her liver tests and severe fatigue improved rapidly. She continues to follow up outpatient and is doing well. DISCUSSION: Sarcoid hepatic granulomas are usually asymptomatic and associated with normal liver function or mild derangement. Fever, interestingly, correlates with hepatic sarcoid, like in our patient. Clinical manifestations of liver involvement are rare and may include chronic cholestasis, portal hypertension or Budd Chiari syndrome. In general, granulomas are found in 4% of all liver biopsy specimens, of these only 13.5%-22% are due to sarcoidosis. An optimal therapeutic regimen for hepatic sarcoidosis remains ill defined. In patients with symptoms or significant hepatic dysfunction, corticosteroids have been used as a first line treatment. Prednisone daily for 8-12 weeks and then gradual tapering over a period of 6-12 months is usually recommended.Figure 1.: Lobular granuloma.Figure 2.: Periportal granuloma.Figure 3.: Periportal granuloma (magnified).