S2842 Painless Jaundice as the Initial Manifestation of Hepatic Sarcoidosis

Abstract

Introduction: Sarcoidosis is a chronic, non-caseating granulomatous inflammatory multi-system disease, most commonly presenting with pulmonary involvement in 90-95% of cases. Only 5-9% of patients present with extrapulmonary involvement in the absence of pulmonary manifestations. Liver involvement is found in 50-70% of patients and is usually asymptomatic and diagnosed incidentally; less than 20% of the patients have a clinically significant form with abdominal pain seen in 15% of patients and jaundice seen in less than 5%. Symptomatic liver involvement is extremely uncommon without pulmonary manifestations. Atypical presentation can lead to underdiagnosis, and if left untreated, can lead to cirrhosis. Here we present a case of painless jaundice without pulmonary symptoms that lead to the diagnosis of hepatic sarcoidosis. Case Description/Methods: A 44-year old female with no significant medical history aside from cholecystectomy presented with 9 lb unintentional weight loss, abdominal distension and yellow eyes. Physical exam was significant for scleral icterus and mild right upper abdominal tenderness. Labs showed Total bilirubin 7.2 mg/dL (unconjugated 0.9). Imaging included an abdominal ultrasound, CT abdomen/pelvis and MRCP, all of which identified hepatosplenomegaly, no biliary dilation, and enlarged upper abdominal, lower mediastinal, pericardial, and retroperitoneal lymph nodes. Laboratory workup for PSC, PBC, ANA, AMA and ANCA were negative. Liver biopsy showed non-necrotizing granulomas, confirming the diagnosis of systemic sarcoidosis. She was subsequently started on steroids which improved her symptoms and biochemical abnormalities. Alkaline phosphatase and bilirubin peaked at 1441 and 8.0 mg/dL and trended down to 1105 and 4.7 mg/dL before discharge. The patient was discharged to have close follow up with gastroenterology and rheumatology. Discussion: Although hepatic sarcoidosis is not a rare entity on its own, her presentation of jaundice and abdominal pain are only seen in less than 5% of cases of sarcoidosis. The absence of pulmonary symptoms makes this presentation particularly rare. Liver biopsy is currently recommended to aide in the diagnosis, which if made early, can lead to early treatment and prevention of advanced liver disease. Thus, it is essential for clinicians to recognize atypical extrapulmonary manifestations in patients without typical pulmonary symptoms. Current treatment recommendations are centered on steroids to which this patient had a promising response.Figure 1.: A - CT Abdomen and Pelvis showing hepatomegaly. B - Chest x ray showing hilar adenopathy Image C - MCRP highlighting lesion in liver.