A C Desbois1, L Biard2, O Addimanda3, M Lambert4, E Hachulla5, D Launay6, F Ackermann7, L Pérard8, A Hot8, F Maurier9, C Mausservey10, F Bernard11, N Noel12, L Alric13, T Mirault14, F Cohen15, S Boussouar16, M Resche-Rigon2, P Cacoub1, D Saadoun17. 1. APHP, Service de Médecine Interne et Immunologie clinique, Groupe Hospitalier Pitié Salpétrière Paris, France; DHU Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie, Paris VI, France. 2. Département de Biostatistiques, Hôpital Saint-Louis, Paris, France. 3. Rheumatology Unit, Department of Internal Medicine, Reggio Emilia, Italy. 4. Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59000 Lille, France; Centre National de Référence des Maladies Autoimmunes et Systémiques Rares (Sclérodermie), France. 5. Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59000 Lille, France; Université de Lille, U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; Centre National de Référence des Maladies Autoimmunes et Systémiques Rares (Sclérodermie), France. 6. Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59000 Lille, France; Université de Lille, U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France; Centre National de Référence des Maladies Autoimmunes et Systémiques Rares (Sclérodermie), France. 7. Service de Médecine Interne, Hôpital Foch, Suresnes, France. 8. Service de Médecine Interne, Groupement Hospitalier-Hôpital Edouard Herriot, Lyon, France. 9. Service de Médecine Interne, Hôpitaux Privés de Metz, France. 10. Service de Médecine Interne, Centre Hospitalier de Chalon Sur Saône, France. 11. Service de Médecine Interne, APHM, Hôpital Nord, Marseille, France. 12. Service de Médecine Interne, APHP, CHU le Kremlin-Bicêtre, France. 13. Service de Médecine Interne, CHU Purpan, UMR 152, IRD Toulouse 3 University, France. 14. Service de Réadaptation Vasculaire, APHP, Hôpital Corentin-Celton, Paris, France. 15. Service de médecine Interne 2, Groupe Hospitalier Pitié-Salpétrière, Paris, France. 16. Service d'Imagerie Cardiovasculaire, Institut de cardiologie, Groupe Hospitalier Pitié-Salpétrière, Paris, France. 17. APHP, Service de Médecine Interne et Immunologie clinique, Groupe Hospitalier Pitié Salpétrière Paris, France; DHU Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie, Paris VI, France. Electronic address: david.saadoun@psl.aphp.fr.
Abstract
OBJECTIVE: To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD). METHODS: A multicenter study evaluating 18 refractory BD patients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents. RESULTS: Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42-62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1). CONCLUSION: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.
OBJECTIVE: To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD). METHODS: A multicenter study evaluating 18 refractory BDpatients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents. RESULTS: Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42-62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1). CONCLUSION: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BDpatients with major vessels disease.
Authors: Emma H Weiss; Christine J Ko; Thomas H Leung; Robert G Micheletti; Arash Mostaghimi; Sarika M Ramachandran; Misha Rosenbach; Caroline A Nelson Journal: Curr Dermatol Rep Date: 2022-03-16