2445 All Liver, No Lungs: An Unusual Presentation of Sarcoidosis

Abstract

INTRODUCTION: Sarcoidosis primarily isolated to the liver—hepatic sarcoidosis (HS)—is rare and typically presents with non-specific gastrointestinal (GI) symptoms, abnormal liver enzyme levels, and radiological findings. We present a patient whom we determined to have HS. CASE DESCRIPTION/METHODS: A 33-year-old woman without significant past medical history presented with 3 months of worsening right upper quadrant pain and 35-40 lb weight loss. She had no other specific complaints. Her exam was notable for epigastric and RUQ tenderness. Her liver was palpable 4 cm below the costal margin. Lab results included Hgb 12.5 g/dL with MCV 79, AST 60, ALT 69, AP 629, and GGT 773. CT scan showed diffuse cyst-like lesions throughout the liver and spleen with peri-portal lymphadenopathy (Figure 1). The chest X-ray was unrevealing. Abdominal ultrasound showed hepatomegaly to 16.1 cm, splenomegaly to 13.1 cm, and periportal and peripancreatic lymphadenopathy. Her viral hepatitis workup, Chromogranin A, CEA, CA-19-9, CA-125, and AFP levels were unremarkable. Autoimmune hepatitis workup was negative. Her angiotensin converting enzyme (ACE) was elevated to 261 IU/L. EGD and colonoscopy were unremarkable, but we took random biopsies. Liver biopsy showed confluent, non-caseating epithelioid granulomas. Biliary destruction with lymphocytic cholangitis was noted (Figure 2). Our gastric biopsies showed non-necrotizing granulomas. We diagnosed her with HS without clinically or radiographically evident lung involvement. Since her symptoms later resolved, we opted against starting steroids and we referred her to the rheumatology and pulmonology services. DISCUSSION: Lung involvement is seen in 90% of sarcoidosis patients, usually in the upper lobes, hilar and mediastinal lymph nodes. The diagnostic criteria for sarcoidosis include clinical and radiographic signs, non-caseating granulomas, and exclusion of other diseases. Elevated ACE levels and non-caseating granulomas are not specific to sarcoidosis and can also be seen in primary biliary cholangitis. What clinched our diagnosis was the gastric biopsies that also showed granulomas. Those with asymptomatic liver disease can be monitored until liver enzymes normalize. Hepatomegaly does not necessitate medical treatment, although patients with weight loss and lymphadenopathy should have prednisone, generally for 12 months. Ursodeoxycholic acid (UDCA) can be given for pruritus and liver enzyme elevations. In advanced HS cases with cirrhosis, liver transplantation is the only recourse.