Hepatic sarcoidosis presenting with portal hypertension without histologically established cirrhosis and reticular hepatic imaging features.
Kenta Yamamoto, Siena Itoh, Kazuki Sahara et al.
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Case in brief
A 50-year-old man was evaluated after non-caseating granulomas were found incidentally on the liver surface during surgery. Tests showed a cholestatic enzyme pattern, mild thrombocytopenia, and elevated angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Imaging showed an irregular liver surface and reticular hypoattenuated structures; endoscopy identified esophagogastric varices. Biopsy demonstrated granulomas in fibrotic portal tracts and spatially heterogeneous portal fibrosis, but no bridging fibrosis, regenerative nodules, or histologically established cirrhosis. He was diagnosed with hepatic and splenic sarcoidosis with portal hypertension.
What made this case unusual
Portal hypertension and reticular hepatic imaging features occurred with portal-predominant, uneven fibrosis but without histologically established cirrhosis; the intervening parenchyma retained contrast uptake on hepatobiliary-phase MRI and post-vascular-phase ultrasonography.
Diagnostic / clinical pearl
In hepatic sarcoidosis, portal hypertension may accompany heterogeneous portal tract fibrosis even when biopsy does not establish cirrhosis; correlate varices and reticular imaging findings with the distribution of fibrosis and granulomas.