ARTHRITIS & RHEUMATISM Vol. 64, No. 8, August 2012, pp 2753–2760 DOI 10.1002/art.34450 © 2012, American College of Rheumatology Immunosuppressants Reduce Venous Thrombosis Relapse in Behc ¸et’s Disease A. C. Desbois, 1 B. Wechsler, 1 M. Resche-Rigon, 2 J. C. Piette, 1 D. Le Thi Huong, 1 Z. Amoura, 1 F. Koskas, 3 K. Desseaux, 2 P. Cacoub, 1 and D. Saadoun 1 Objective. To investigate and describe the long- term outcome of venous thrombosis in patients with Behc ¸et’s disease (BD). Methods. In a retrospective cohort of 807 BD patients, a reported 296 patients (36.7%) (73.3% male, median age 30 years [interquartile range 24–36 years]) met the international classification criteria for BD and had venous thrombosis. We assessed factors associated with thrombosis relapse and mortality. Results. There were a total of 586 venous throm- bosis events, including 560 cases of deep thrombosis and 26 cases of superficial thrombosis. Deep venous throm- bosis events included 323 cases of limb thrombosis (55.1%), 77 cases of cerebral venous thrombosis (13.1%), 57 cases of pulmonary embolism (9.7%), 63 cases of vena cava lesions (10.7%), 14 cases of Budd- Chiari syndrome (2.4%), and 13 cases of cervical vein thrombosis (2.2%). One hundred of 296 patients (33.8%) experienced at least 1 venous thrombosis re- lapse. The mortality rate was 6.4% (19 of 296 patients) after a median followup of 4.75 years (interquartile range 2–7 years). In univariate analysis, death was associated with cardiac involvement (P  0.026) and Budd-Chiari syndrome (P  0.004). In multivariate analysis, the use of immunosuppressive agents was found to prevent relapse of venous thrombosis (hazard ratio 0.27 [95% confidence interval 0.14–0.52], P  0.00021), and there was a trend toward prevention of relapse with the use of glucocorticoids (hazard ratio 0.62 [95% confidence interval 0.40–0.97], P  0.058). Conclusion. Immunosuppressive agents signifi- cantly reduce venous thrombosis relapse in BD. Behc ¸et’s disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous sys- tem manifestations (1). BD is included in the wide spectrum of vasculitis. Vasculitis is a principal pathologic finding in BD, and vessels of all sizes are involved, both in the arterial and venous systems. Large-vessel vasculi- tis is not a rare manifestation of BD (2,3). The preva- lence of large-vessel vasculitis varies according to the findings of investigators and the populations under study. Venous disease is more common than arterial involvement, and its reported prevalence may account for 14–39% of patients with BD (4–9). Venous throm- bosis in BD is a severe disorder that may affect many different sites including the inferior vena cava, superior vena cava, pulmonary artery, suprahepatic vessels, and cardiac cavities. We previously reported that 17% of deaths in BD were caused by venous involvement, mainly pulmonary embolism or Budd-Chiari syndrome (10). Treatment of venous thrombosis in BD is not well defined. Treatment modalities for thrombosis include glucocorticoids and other immunosuppressive agents (azathioprine, cyclosporine, cyclophosphamide), antico- agulation therapy, and intravenous thrombolysis. Inter- ventional procedures such as filter insertion or throm- bectomy have also been reported (11). However, there are no large controlled studies regarding the best ap- proach for BD patients with thrombosis complications, 1 A. C. Desbois, MD, B. Wechsler, MD, J. C. Piette, MD, D. Le Thi Huong, MD, Z. Amoura, MD, P. Cacoub, MD, D. Saadoun, MD, PhD: UMR CNRS 7211, INSERM U959, Groupe Hospitalier Pitie ´-Salpe ˆtrie `re, AP-HP, and Universite ´ Pierre et Marie Curie–Paris 6, Paris, France; 2 M. Resche-Rigon, MD, PhD, K. Desseaux, MD: INSERM U717, Ho ˆpital Saint-Louis, AP-HP, Paris, France; 3 F. Kos- kas, MD, PhD: Groupe Hospitalier Pitie ´-Salpe ˆtrie `re, AP-HP, and Universite ´ Pierre et Marie Curie–Paris 6, Paris, France. Address correspondence to D. Saadoun, MD, PhD, Ho ˆpital Pitie ´-Salpe ˆtrie `re, AP-HP, Service de Me ´decine Interne et UMR CNRS 7211, INSERM U959, 83 Boulevard de l’Ho ˆpital, Paris F-75013, France. E-mail: david.saadoun@psl.aphp.fr. Submitted for publication July 18, 2011; accepted in revised form February 16, 2012. 2753