Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis

  • Rym Ennaifer | rym.ennaifer@yahoo.fr Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
  • Dhouha Bacha Faculty of Medicine, University of Tunis El Manar, Tunis; Department of Pathology, Mongi Slim Hospital, Tunis, Tunisia.
  • Hayfa Romdhane Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
  • Myriam Cheikh Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
  • Houda Ben Nejma Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
  • Najet BelHadj Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.

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.

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Published
2015-12-21
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Section
Case Reports
Keywords:
Sarcoidosis, Budd-Chiari syndrome, liver.
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How to Cite
Ennaifer, R., Bacha, D., Romdhane, H., Cheikh, M., Ben Nejma, H., & BelHadj, N. (2015). Budd-Chiari syndrome: an unusual presentation of multisystemic sarcoidosis. Clinics and Practice, 5(3). https://doi.org/10.4081/cp.2015.768