2489 A Rare Case of Isolated Symptomatic Hepatic Sarcoidosis

Abstract

INTRODUCTION: Sarcoidosis is an inflammatory granulomatous disease that has multi- organ involvement with varied course of disease expression and progression. Liver involvement is seen in only a hand full of cases, especially with florid symptoms. Here, we present a rare case of symptomatic isolated hepatic sarcoidosis mimicking malignancy, that was promptly diagnosed. CASE DESCRIPTION/METHODS: A 60- year old African American female, who is otherwise healthy, presented to our tertiary care center with a month-long history of worsening epigastric pain, nausea, vomiting, malaise, weight loss and night sweats. Exam was remarkable for hepatomegaly and labs significant for elevated ALP and GGT, mild transaminitis and elevated CA 19-9 levels. CT scan revealed hypodensities and perfusion defects throughout the liver, hepatosplenomegaly and hilar lymphadenopathy, which had been confirmed by MRI and Triple-phase CT of the liver. With the suspicion for malignancy, liver biopsy was pursued and showed epithelioid-cell granulomas with focal necrosis and multiple asteroid bodies, pathognomonic for sarcoidosis. Subsequently, ACE levels were found to be elevated. Patient was then started on prednisone and immunosuppressants with relief of symptoms. A repeat CT scan 6-months later, showed resolution of liver lesions and improvement in liver chemistries. DISCUSSION: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Per ACCESS study, liver involvement of sarcoidosis was described in only 11.5% of 736 patients, more commonly seen in African American population than Caucasian, with only 10% of them were reported to have transaminitis. Hepatic involvement appears to be clinically silent with most patients presenting with constitutional symptoms, even then liver lesions on CT are described in only 5% of the patients. Steroid therapy and immunomodulators are used to help with symptom control, however their effect on the natural progression of the disease is not established. Our patient presentation with significant symptoms and imaging findings, prompted us to think beyond the obvious. Sarcoidosis is a diagnosis of exclusion; hence it is essential for clinicians to have a high degree of suspicion for hepatic involvement.

Identification of Isolated Hepatic Sarcoidosis With 18F-FDG PET/CT and MRI

“…Hepatic involvement of systemic sarcoidosis is relatively common, and although it is usually asymptomatic, it can rarely lead to complications including cirrhosis and portal hypertension 1–5 . Isolated hepatic involvement of sarcoidosis is very rare 6–8 …”