Sarcoidosis Masquerading as Long-Standing Cholestasis

Abstract

Hepatic sarcoidosis is an exceedingly rare extrapulmonary manifestation of sarcoidosis, with the majority remaining stable for years without clinical clues, only displaying biochemical abnormalities. Amongst the literature, the timeline to cirrhosis has not been parsed out; hepatomegaly develops in 50% and cirrhosis in 33% of all hepatic sarcoidosis patients, making this an essential issue in this patient population. Interestingly, the risk for hepatocellular carcinoma remains high regardless of cirrhosis development. Corticosteroids and biologics remain the mainstay of therapy, although refractory cases may require deeper immunosuppression. Liver transplantation is seen in a handful of cases with promising results. We present an interesting case of cholestatic pattern livery injury in our outpatient setting that was eventually discovered to be hepatic sarcoidosis. Mild biochemical derangements or sole elevations in alkaline phosphatase are under-recognized, and patients often progress to cirrhosis and end-stage liver disease. This diagnostic miss has significant implications and represents an opportunity to treat liver disease with a reversible cause. Consensus guidelines recommend alkaline phosphatase screening in newly diagnosed cases of sarcoidosis.

Keywords: Cholestasis; Extrapulmonary; Liver; Sarcoid; Transaminitis.

Figure 1

Computed tomography (CT) of the abdomen revealing changes consistent with cirrhosis and splenomegaly.

Figure 2

Hematoxylin and eosin-stained histological examination at × 20 magnification revealing non-caseating granulomas (arrows) in the left lower lung.

Figure 3

Hematoxylin and eosin-stained histological examination at × 40 magnification revealing epithelioid histiocytes, lymphocytes, and multinucleated giant cells in the left lower lung.

Figure 4

Diff-Quik stained histological examination at × 20 magnification revealing epithelioid histiocytes and lymphocytes (arrows) from the left hilar lymph nodes.