Intractable Rare Dis Res. 2019;8(1):60-66. (DOI: 10.5582/irdr.2018.01128)
Budd-Chiari Syndrome in BehÃ§et's Disease successfully managed with immunosuppressive and anticoagulant therapy: A case report and literature review.
Oblitas CM, Galeano-Valle F, Toledo-Samaniego N, Pinilla-Llorente B, Del Toro-Cervera J, Ãlvarez-Luque A, GarcÃa-GarcÃa A, Demelo-Rodriguez P
BehÃ§et's Disease (BD) is a rare, chronic and recurrent inflammatory multisystemic condition of unknown origin that can affect any tissue. The vascular system is involved in 5-40% of cases of BD, including venous and arterial beds and it has a relapsing course. Budd-Chiari syndrome (BCS) is a rare complication of BD with a frequency of < 5% among patients with vascular involvement and is more frequent in men (89.5%). Two clinical presentation groups of BCS related to BD have been described: the "symptomatic" form and the "silent" form. We present a case of BD in a young woman presented as symptomatic severe BCS with rapid progression of coagulopathy reaching a spontaneous INR of 1.74 and increased ascites by ultrasound control. BD was confirmed through clinical history. The patient was treated with a high-dose pulse of corticosteroids and cyclophosphamide with a strikingly favorable response in the first forty-eight hours. Although several studies have demonstrated a survival improvement with the use of transjugular intrahepatic portosystemic shunt in patients with severe BCS, it was discarded due to the lack of evidence of this procedure in patients with BD and the fact that it could trigger a vascular pathergy phenomenon. Vascular BD should be suspected in recurrent venous and/or arterial thrombosis since it is associated with high morbidity and mortality. Immunosuppressive treatment is critical for the management of vascular involvement in BD. However, the role of anticoagulation is debatable. We suggest an algorithm for the management of BCS associated with BD.