J Yeungnam Med Sci.  2022 Jul;39(3):256-261. 10.12701/yujm.2021.01151.

Primary hepatic sarcoidosis presenting with cholestatic liver disease and mimicking primary biliary cholangitis: a case report

• Park YJ
• Woo HY
• Kim MB
• Ahn J
• Heo J

• KMID: 2532080
• DOI: http://doi.org/10.12701/yujm.2021.01151

Abstract

Sarcoidosis often involves the liver. However, primary hepatic sarcoidosis confined to the liver without evidence of systemic involvement is rare. We report the case of a 37-year-old man with hepatic sarcoidosis who initially presented with elevated liver enzymes and suspicious cirrhotic nodules on computed tomography. The patient had cirrhosis but did not have portal hypertension. Based on the initial histopathologic finding of chronic granulomatous inflammation and the common clinical characteristics of sarcoidosis, he was initially diagnosed with primary biliary cholangitis, and his daily dosage of ursodeoxycholic acid was increased to 900 mg. After 14 months of treatment, his total serum bilirubin concentration was 10.9 mg/dL (upper normal limit, 1.2 mg/dL). Additionally, a transjugular liver biopsy revealed multiple noncaseating granulomas. He was diagnosed with primary hepatic sarcoidosis involving the lungs, heart, spleen, kidneys, and skin. Treatment with methylprednisolone was initiated. Two weeks later, he was started on azathioprine, and the dose of steroid was simultaneously reduced. These findings indicate the importance of including hepatic sarcoidosis as a possible diagnosis in patients with elevated liver enzymes or cryptogenic cirrhosis.

Keyword

Biliary tract; Cholestasis; Liver cirrhosis; Sarcoidosis

Figure

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  Fig. 1. Initial abdominal computed tomography (CT) and chest X-ray of the patient. (A) Enhanced CT image of the abdomen in the portal-venous phase, showing underlying liver cirrhosis with multiple cirrhotic nodules in both lobes of the liver. (B) The first chest X-ray shows no evidence of hilar lymphadenopathy.

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  Fig. 2. Histopathology of the first transjugular liver biopsy. (A) The liver shows chronic granulomatous inflammation without necrosis. (B) Focal aggregates of epithelioid histiocytes are present (hematoxylin and eosin stain; [A] ×200, [B] x400).

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  Fig. 3. Clinical and histologic findings of the skin. (A) Erythematous plaques are present on the right elbow and left knee. (B) The skin shows noncaseating granulomas composed of epithelioid histiocytes (hematoxylin and eosin stain, ×200).

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  Fig. 4. Histopathology of the second transjugular liver biopsy. (A, B) The liver shows multiple noncaseating granulomas in the hepatic capsule and portal tracts (hematoxylin and eosin stain; A, ×200; B, ×400).

Reference

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