title: Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients.
abstract: The vasculitis of Behçet disease (BD) is distinctive because of involvement of both arteries and veins of all sizes. The concept of vasculo-Behçet disease has been adopted for cases in which vascular manifestations are present and often dominate the clinical features. While venous manifestations are frequent and have been reported in many publications, data regarding arterial lesions in patients with BD are rare and often isolated. In this study, we report the main characteristics, treatment, and long-term outcome of 101 patients with arterial lesions among a cohort of 820 (12.3%) BD patients. Factors that affect prognosis were assessed by multivariate analysis. There were 93 (91.2%) male patients; the median (Q1-Q3) age at diagnosis of BD was 33 (27-41) years. Arterial lesions included aneurysms (47.3%), occlusions (36.5%), stenosis (13.5%), and aortitis (2.7%). Lesions mainly involved the aorta (n = 25) and femoral (n = 23) and pulmonary (n = 21) arteries. Patients with arterial lesions were more frequently male (91.2% vs. 62.4%, respectively; p = 0.017) and had higher rates of venous involvement (80.4% vs. 29.8%, respectively; p < 0.001) compared to patients without arterial manifestations. Thirty-nine (38.6%) patients achieved complete remission. In multivariate analysis, the presence of venous involvement (odds ratio [OR], 0.29; 95% confidence interval [CI], 0.08-1.11) and arterial occlusive lesions (OR, 0.13; 95% CI, 0.01-1.25) were negatively associated with complete remission. The use of immunosuppressants (OR, 3.38; 95% CI, 0.87-13.23) was associated with the occurrence of complete remission. The 20-year survival rate was significantly lower in BD patients with arterial involvement than in those without arterial lesions (73% vs. 89%, respectively; p < 0.0001). In conclusion, the long-term outcome of arterial lesions in BD is poor, especially in the case of occlusive lesions and associated venous involvement. The use of immunosuppressants improved the prognosis.
authors.affiliation: From Department of Internal Medicine (DS, BA, BW, GG, JCP, DLTH, ZA, P. Cacoub) and Laboratory I3 (DS) "Immunology, Immunopathology, Immunotherapy,'' UMR CNRS 7211, INSERM U959; Department of Radiology (P. Cluzel); and Department of Vascular Surgery (FK); Groupe Hospitalier Pitié-Salpêtrière, Université Pierre et Marie Curie, Paris 6, Paris; Department of Biostatistics and Medical Data Processing (KD, MRR); INSERM U717, Hôpital Saint-Louis, Paris, France; and Department of Internal Medicine (HH, TBS), Hôpital La Rabta, Tunis, Tunisia.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Bouchra
authors.initials: B
authors.lastname: Asli
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Habib
authors.initials: H
authors.lastname: Houman
authors.forename: Guillaume
authors.initials: G
authors.lastname: Geri
authors.forename: Kristel
authors.initials: K
authors.lastname: Desseaux
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Du Le Thi
authors.initials: DLT
authors.lastname: Huong
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Tara Ben
authors.initials: TB
authors.lastname: Salem
authors.forename: Philippe
authors.initials: P
authors.lastname: Cluzel
authors.forename: Fabien
authors.initials: F
authors.lastname: Koskas
authors.forename: Mathieu
authors.initials: M
authors.lastname: Resche-Rigon
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001158:Arteries / Q000473:pathology; D001167:Arteritis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D001528:Behcet Syndrome / Q000150:complications* / Q000188:drug therapy / Q000401:mortality / Q000473:pathology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D016017:Odds Ratio; D012074:Remission Induction; D012189:Retrospective Studies; D012737:Sex Factors; D018709:Statistics, Nonparametric; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents