The Great Mimicker Strikes Again: Isolated Hepatic Sarcoidosis

Abstract

Sarcoidosis is a multisystem disorder of idiopathic origin with formation of noncaseating granulomas. Pulmonary findings are evident in over 85% of the cases. Extrapulmonary symptoms are generally seen in the skin, eyes, heart, and CNS. Although hepatic granulomas are present in 50-60% of cases, patients presenting initially with isolated ascites is uncommon. A 43 year old Caucasian male with a history of HTN, CAD, ESRD on hemodialysis, and CVA presented with complaint of constant abdominal discomfort associated with nausea/vomiting. Physical exam revealed abdominal distention. Labs revealed abnormal liver chemistries including an Alkphos 388 U/L, total bilirubin 1.4 mg/dL, total protein 6.5 g/dL, albumin 1.9 g/dL, revealing an elevated gamma gap (4.6). Ultrasound demonstrated large ascites and splenomegaly. Doppler evaluation of hepatic veins showed no evidence of venous hypertension or thrombosis. Ascites sampling resulted an albumin level of 1.8 with low calculated serum-albumin ascites gradient (SAAG) of 0.1. The patient clinically improved after therapeutic paracentesis and was discharged. 6 months later, he returned with SOB, fatigue, and abdominal discomfort. He was mildly leukopenic (WBC 3.4x103 u/L) and a chest CT revealed a left lower lobe consolidation with mediastinal lymphadenopathy. Abdominal CT demonstrated splenomegaly (17cm), patent portal and hepatic veins with normal appearing liver. However, our gastroenterology consultants performed a liver biopsy due to the presence of a low SAAG with high protein ascites. Microscopic evaluation of the liver biopsy revealed numerous small non-necrotizing granulomas and iron staining positive showed kupffer cells confirming hepatic sarcoidosis. Steroid therapy was initiated and our patient rapidly improved. This case highlights the importance of keeping a high suspicion for sarcoidosis on the differential when liver disease is clinically present and there is a low SAAG and high protein ascites present. Symptomatic hepatic involvement only occurs in 5 to 15 percent of sarcoid cases but a SAAG below 1 should raise suspicion. Liver biopsy as performed for our patient is required to substantiate the clinical suspicion. Untreated, patients can have a rapid clinical decline. Prompt therapy with glucocorticoids treatment is considered first-line therapy with concomitant ursodeoxycholic acid for cholestasis. In conclusion, a thorough work-up and diagnosis are mandatory when low SAAG ascites is encountered.2330_A Figure 1. Indications for Liver Biopsy2330_B Figure 2. Granulomatous Hepatitis Ref: Chan, Anthony2330_C Figure 3. Hepatic Granuloma Ref: Chan, Anthony Pathology Outlines