Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis.

Rym Ennaifer,Dhouha Bacha,Najet Belhadj,Myriam Cheikh,Houda Ben Nejma,Hayfa Romdhane

doi:10.4081/cp.2015.768

Rym Ennaifer, Dhouha Bacha + Show 4 more

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https://doi.org/10.4081/cp.2015.768

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Abstract

Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.

Highlights

Introduction pleted with acomputed tomography (CT) scan of the chest, which failed to ascertain an underlying prothrombotdemonstrated mediastinal and hilar ic condition, the diagnosis of Budd-Chiari syndrome (BCS) complicatadenopathies with multiple micronodular ing sarcoidosis was the most likely
Liver involvement is common but is rarely granulomas consisting of a compact aggregate We reported a case of multisystemic sarsymptomatic.[3]
A minority of patients will (BCS) related to hepatic sarcoidosis have been more frequent in portal tracts

Summary

Introduction

Introduction pleted with aCT scan of the chest, which failed to ascertain an underlying prothrombotdemonstrated mediastinal and hilar ic condition, the diagnosis of BCS complicatadenopathies with multiple micronodular ing sarcoidosis was the most likely. A few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic o sarcoidosis have been described so far.

Results

Conclusion