Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study

• Published in: Clinical rheumatology Vol. 41; no. 1; pp. 177 - 186
• Main Authors: Akyol, Lütfi, Toz, Bahtiyar, Bayındır, Özün, Zengin, Orhan, Cansu, DöndüÜsküdar, Yiğit, Murat, Çetin, Gözde Yıldırım, Omma, Ahmet, Erden, Abdulsamet, Küçükşahin, Orhan, Altuner, Mehmet Şakir, Çorba, BurçinŞeyda, Ünal, Ali Uğur, Küçük, Hamit, Küçük, Adem, Balkarli, Ayşe, Gönüllü, Emel, Tufan, Ayşe Nur, Bakırcı, Sibel, Öner, Sibel Yılmaz, Balcı, Mehmet Ali, Kobak, Şenol, Yazıcı, Ayten, Özgen, Metin, Şahin, Ali, Koca, Süleyman Serdar, Erer, Burak, Gül, Ahmet, Aksu, Kenan, Keser, Gökhan, Onat, Ahmet Mesut, Kısacık, Bünyamin, Kaşifoğlu, Timuçin, Çefle, Ayşe, Kalyoncu, Umut, Sayarlıoğlu, Mehmet
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 2022; Springer Nature B.V
• Subjects: Anticoagulants; Behcet Syndrome - complications; Behcet Syndrome - drug therapy; Behcet Syndrome - epidemiology; Behcet's syndrome; Budd-Chiari Syndrome - complications; Budd-Chiari Syndrome - epidemiology; Cohort Studies; Corticosteroids; Diagnosis; Humans; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Interferon; Medicine; Medicine & Public Health; Mortality; Original Article; Patients; Prognosis; Retrospective Studies; Rheumatology; Thrombosis; Vein & artery diseases; Vena Cava, Inferior; Budd–Chiari syndrome; Prognosis; Behçet’s disease; Thrombosis; Inferior vena cava
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-021-05878-2

Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS ( p  = 0.005 and p  = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p  < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls ( p  = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.