University of Hawaii School of Medicine Honolulu, HI, United States
Eric Wien, MD1, Frederic E. Wien, MD2 1University of Hawaii School of Medicine, Honolulu, HI; 2Hackensack University Medical Center Meridian School of Medicine, Paterson, NJ
Introduction: Sarcoidosis is a multisystemic inflammatory disorder characterized by the presence of non-caseating granulomas. More classically with pulmonary involvement, sarcoidosis can affect any organ, with hepatic involvement in up to 70% of patients. We present a case of biopsy confirmed hepatic sarcoidosis initially presenting as suspected primary biliary cirrhosis (PBC).
Case Description/Methods: A 52-year-old female with H pylori negative gastritis, GERD, diverticulitis status post colonic resection and prior alcohol use presented with nausea, abdominal pain, and pruritis in the setting of elevated alkaline phosphatase and mildly elevated transaminases. She denied fever, jaundice, shortness of breath, cough, or chest pain. Physical examination revealed a nontender abdomen and no skin lesions. The liver was 12cm in the MCL and non tender to palpation and no splenomegaly noted. Labs: ALT 77 IU/L, AST 71 IU/L, Alk Phos 698 IU/L, GGP 776 IU/L, total bilirubin 0.7 mg/dl, AMA and anti-SMA negative. Ultrasound reported liver tissue heterogeneity with fibrosis and no choledocholithiasis or cholelithiasis. A liver biopsy revealed noncaseating granulomas and fibrosis consistent with sarcoidosis and early cirrhosis. There was no evidence of florid duct lesions of PBC. Serum angiotensin converting enzyme was 131 U/L (N= 9-67 U/L). CT Chest showed mildly enlarged mediastinal/hilar lymph nodes with scattered tree-in-bud/reticular opacities, which were likely inflammatory. The patient received Ursodeoxycholic Acid (UDCA) and is being followed for possible use of corticosteroids.
Discussion: Given its often asymptomatic presentation, hepatic sarcoidosis is underdiagnosed as only 5-30% of patients present with abdominal pain, jaundice, nausea, vomiting or hepatosplenomegaly. While initial workup suspected PBC, hepatic sarcoidosis is also known to present with elevated alkaline phosphatase and diffuse pruritis. With the asymptomatic nature of this disease and the risk of medication adverse effects, most cases of hepatic sarcoidosis do not require treatment. Therapy with UDCA may limit liver fibrosis and improve intrahepatic cholestasis. Corticosteroids and other immunosuppressive agents may be given in more severe cases. Liver transplantation is rarely required and is reserved for advanced hepatic sarcoidosis. Though often asymptomatic, hepatic sarcoidosis can cause symptoms secondary to severe organ damage. Underdiagnosed, a high clinical suspicion is required to make the diagnosis.
Figure: H&E stain of liver with numerous large noncaseating epithelioid granulomas with tightly packed epithelioid cells present in the portal triads and some in the hepatic lobules. Figure 1 also shows a focus of an asteroid body, stellate inclusion within giant cells.
Eric Wien indicated no relevant financial relationships.
Frederic Wien indicated no relevant financial relationships.
Eric Wien, MD1, Frederic E. Wien, MD2. P1861 - Hepatic Sarcoidosis Presenting as Suspected Primary Biliary Cirrhosis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.