title: [Cardiovascular involvement in Behçet's disease].
abstract: Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France; DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, AP-HP, 75013 Paris, France.
authors.forename: A-C
authors.initials: AC
authors.lastname: Desbois
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Wechsler
authors.affiliation: Service de radiologie, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Cluzel
authors.affiliation: Service de cardiologie, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Helft
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Boutin
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France.
authors.forename: J-C
authors.initials: JC
authors.lastname: Piette
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France; DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, AP-HP, 75013 Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Service de médecine interne, centre de référence des maladies autoimmunes rares, groupe hospitalier Pitié-Salpêtrière, 75013 Paris, France; DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, AP-HP, 75013 Paris, France. Electronic address: david.saadoun@psl.aphp.fr.
authors.forename: D
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D002318:Cardiovascular Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011379:Prognosis; D020246:Venous Thrombosis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D000925:Anticoagulants; D007166:Immunosuppressive Agents
keywords: Atteinte cardiaque; Atteinte vasculaire; Behçet's disease; Cardiac involvement; Maladie de Behçet; Vascular involvement; Vascularite; Vasculitis