11 October 2018 : Case report
Challenging differential diagnosis, Rare diseaseAbdisamad M. Ibrahim1ABCDEFG*, Bishal Bhandari1BDEFG, Paolo K. Soriano1ACDEFG, Zafar Quader2ABCDFG, John Z. Gao3BDEG, Dmitry Shuster4ACDEFG, Chaitanya K. Mamillapalli5AEFG
Am J Case Rep 2018; 19:1212-1215
BACKGROUND: Sarcoidosis is a systemic disease that can affect any organ, including the liver. It is manifested by the presence of non-caseating granulomas within involved organs, most commonly the pulmonary, lymphatic, and hepatic system. Unlike pulmonary or lymphatic involvement, hepatic involvement is usually asymptomatic and it is underdiagnosed. Here, we report a case of a patient with a history of pulmonary sarcoidosis who developed hepatic sarcoidosis.
CASE REPORT: 68-year-old female with pulmonary sarcoidosis with a 2-week history of severe abdominal pain and epigastric tenderness presented to our center. Abdominal magnetic resonance imaging (MRI) demonstrated mild hepatic fibrosis and cirrhosis. A thorough workup was performed including a liver biopsy which showed chronic non-necrotizing granulomas consistent with sarcoidosis. She was started on prednisone and subsequently improved. The patient was symptom-free on follow-up 1 month later.
CONCLUSIONS: The majority of patients with hepatic sarcoidosis are usually asymptomatic, with only 5–30% presenting with abdominal pain, jaundice, nausea, vomiting, and hepatosplenomegaly. In rare cases, hepatic sarcoidosis can lead to cholestasis, portal hypertension, cirrhosis, or Budd-Chiari syndrome. Treatment with steroids is the mainstay of therapy; however, in severe cases, patients may require liver transplantation. This case report demonstrates that hepatic sarcoidosis is a serious condition, and if not treated, can lead to portal hypertension and cirrhosis. In patients with sarcoidosis, early detection and longitudinal follow-up is important in preventing overt liver failure.
Keywords: Cholestasis, Liver Cirrhosis, sarcoidosis
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