Abstract

Sarcoidosis is a multisystem disease usually affecting the chest, hilar lymph nodes, and lungs, but can potentially involve any organ; therefore, its clinical presentation may vary. Hepatobiliary involvement is rare, and typically asymptomatic; however, it can lead to cirrhosis, and may require liver transplantation. In this report, we present a rare case of a patient affected by sarcoidosis with hepatobiliary involvement. He presented to our hospital complaining of dyspnea triggered by moderate efforts and oppressive thoracic discomfort. Chest X-ray showed multiple bilateral nodular opacities and enlargement of both hilar regions, confirmed by a subsequent total-body computed tomography scan and positron emission tomography, which also revealed cardiac, splenic, and hepatic involvement. Liver function was studied via gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging, and magnetic resonance cholangiopancreatography (MRCP) was also performed. The diagnosis of sarcoidosis was finally achieved via liver biopsy, revealing non-necrotizing granulomas in the periportal space. The patient was treated with prednisone per os, with regression of all lesions at all levels. Although other cases of biliary sarcoidosis have been described, this report provides a complete image set of Gd-EOB-DTPA-enhanced magnetic resonance and MRCP images that is lacking in the English literature, and which may be useful for diagnosis.

Highlights

  • Sarcoidosis is a multisystem inflammatory disease that generally affects patients aged 40–55 years, without significant gender differences, and with a variable incidence rate based on the country and ethnic group [1]

  • To the best of our knowledge, in the scientific literature, there is a lack of reported cases presenting a complete set of magnetic resonance imaging (MRI) images, which could guide the radiologist to a confident diagnosis of hepatobiliary sarcoidosis, even in asymptomatic patients with negative liver laboratory tests

  • The present study describes a case of hepatic sarcoidosis involving the biliary tract investigated via Computed tomography (CT), magnetic resonance cholangiopancreatography (MRCP), and functional MRCP performed after infusion of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)

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Summary

Introduction

Sarcoidosis is a multisystem inflammatory disease that generally affects patients aged 40–55 years, without significant gender differences, and with a variable incidence rate based on the country and ethnic group [1]. Enlargement of the liver and spleen can be found in half of patients—especially in the presence of portal hypertension The latter is reported to cause esophageal varices in up to 78% of cases, rather than severe liver dysfunction, which is an unusual complication. To the best of our knowledge, in the scientific literature, there is a lack of reported cases presenting a complete set of MRI images, which could guide the radiologist to a confident diagnosis of hepatobiliary sarcoidosis, even in asymptomatic patients with negative liver laboratory tests. The present study describes a case of hepatic sarcoidosis involving the biliary tract investigated via CT, magnetic resonance cholangiopancreatography (MRCP), and functional MRCP performed after infusion of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)

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