S3210 Unraveling Extrapulmonary Sarcoidosis in a Patient With Unexplained Cholestatic Liver Injury

Abstract

Introduction: Sarcoidosis is an autoimmune condition characterized by non-caseating granulomas most commonly in the lungs and hilar lymph nodes. We report a case of sarcoidosis presenting as hepatomegaly and cholestatic liver injury. Case Description/Methods: A 46-year-old female with end stage renal disease, latent tuberculosis (TB) and daily use of herbal teas, who had multiple hospitalizations over a 6-month period with similar presentation, presented with right upper quadrant pain and jaundice. Examination revealed pulsatile abdomen and jugular venous distention. During each admission, she had elevated alkaline phosphatase and total bilirubin to a peak of 2003 U/L and 37.4 mg/dL respectively, with otherwise normal liver enzymes. Infectious workup and tumor markers were negative. On her second admission, she had rapid improvement in her lab abnormalities and symptoms after initiating steroids for treatment of Bell’s palsy, otherwise, on other admissions she had slow improvement with ursodeoxycholic acid. Initial ultrasound abdomen, magnetic resonance cholangiopancreatography, and triphasic computed tomography (CT) liver showed hepatomegaly, otherwise unremarkable. She had two liver biopsies which revealed sinusoidal congestion and dilation, hepatocellular and sinusoidal fibrosis consistent with chronic venous outflow obstruction and few non-necrotizing granulomas. Repeated transthoracic echocardiogram eventually showed mild tricuspid and pulmonary insufficiency. Right heart catheterization showed mild pulmonary hypertension and hemodynamics not suggestive of a cardiac cause of liver disease. In a last attempt to locate tissue for biopsy, a CT chest, abdomen and pelvis was obtained and showed airspace opacities within the left lower lobe likely related to inflammatory process and an area of enhancement at the junction of segment 5 and 6 of the liver that could relate to hepatic sarcoid. She was started on Prednisone 40mg daily for presumed hepatic sarcoid and Rifampin 600mg daily for latent TB. She will undergo bronchoscopy with biopsy and renal biopsy to further establish a unifying diagnosis (Figure). Discussion: After an extensive workup was found to be negative, sarcoidosis became the top differential given non-caseating granulomas on liver biopsy, nerve palsy, renal disease, mild pulmonary hypertension and response to steroids. Lack of pulmonary involvement is an uncommon presentation in sarcoidosis, occurring in 5-9% of cases overall. Hepatic sarcoidosis occurs in 11-80% of cases and is mostly asymptomatic.Figure 1.: Hepatomegaly with heterogeneous enhancement. There is an area of increased enhancement at the junction of segment 5 and 6 (arrow) of the liver which is nonspecific, but could relate to hepatic sarcoidosis given clinical picture.