Article

Budd-Chiari syndrome complicating hepatic sarcoidosis: definitive treatment by liver transplantation: a case report.

Department of Pathology, CHU de Liège, University of Liège, B4000 Liège, Belgium.
Transplantation Proceedings (Impact Factor: 0.95). 10/2009; 41(8):3432-4. DOI: 10.1016/j.transproceed.2009.09.021
Source: PubMed

ABSTRACT Sarcoidotic involvement of the liver is frequent, albeit uncommonly symptomatic. Severe complications are rare, but may seldom require liver transplantation. Budd-Chiari syndrome has been described in a few patients with hepatic sarcoidosis. Herein we have reported the case of a young woman suffering from hepatic sarcoidosis who developed severe cholestasis and chronic Budd-Chiari syndrome. She successfully underwent orthotopic liver transplantation (OLT) and is asymptomatic with normal liver function at 3 years follow-up. Histopathological assessment of the liver explant demonstrated a florid granulomatous process, with involvement of the large intrahepatic veins, providing an anatomical basis for the vascular flow disturbances. This case adds further evidence that liver transplantation may be the curative treatment for complicated sarcoidotic liver disease.

0 Bookmarks
 · 
134 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sarcoidosis may affect functional outcome and sometimes may be a life-threatening condition. Functional outcome is particularly compromised by cutaneous, ocular, nasal, osteoarticular manifestations and some neurological expression. Central nervous system involvement and cardiac sarcoidosis could be life-threatening, as laryngeal and renal involvement. Diagnosis is sometimes very difficult to obtain. Several tissue biopsies are usually necessary to rule out all differential diagnosis. Extrathoracic manifestations of severe sarcoidosis require a corticosteroid therapy, initiated, if life-threatening at a daily dose of 1 mg/kg, usually associated with an other immunosuppressive drug (methotrexate, cyclophosphamide, anti TNFα).
    La Revue de Médecine Interne 02/2011; 32(2):80-85. · 1.32 Impact Factor
  • Annals of Hematology 04/2013; 92(12). · 2.40 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1 PH), in group 5 conditions is also discussed.
    Clinics in chest medicine 12/2013; 34(4):753-778. · 2.51 Impact Factor