| Title: |
Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: french multicenter case-control study |
| Authors: |
Roupie, Anne Laure; Guedon, Alexis; Terrier, Benjamin; Lahuna, Constance; Jachiet, Vincent; Regent, Alexis; De Boysson, Hubert; Carrat, Fabrice; Seguier, Julie; Terriou, Louis; Versini, Mathilde; Queyrel, Viviane; Groh, Matthieu; Benhamou, Ygal; Maurier, Francois; Ledoult, Emmanuel; Clech, Lenaig Le; D''''aveni, Maud; Rossignol, Julien; Galland, Joris; Willems, Lise; Jourde-Chiche, Noémie; Peterlin, Pierre; Roux-Sauvat, Marielle; Parcelier, Anne; Wemeau, Mathieu; Lambert, Marc; Belizna, Cristina; Puechal, Xavier; Swiader, Laure; Cohen-Valensi, Rolande; Noc, Valerie; Dao, Emmanuel; Thepot, Sylvain; De Fremont, Gregoire Martin; Tanguy-Schmidt, Aline; Koka, Anne Marfaing; Bussone, Guillaume; Philipponnet, Carole; Konate, Amadou; Cavaille, Guilhem; Guilpain, Philippe; Allain, Jean-Sebastien; Broner, Jonathan; Solary, Eric; Ruivard, Marc; De Renzis, Benoit; Corm, Selim; Baati, Nadia; Schleinitz, Nicolas; Ponsoye, Matthieu; Stamatoullas-Bastard, Aspasia; Ades, Lionel; Dellal, Azeddine; Tchirkov, Andrei; Aouba, Achille; Fenaux, Pierre; Fain, Olivier; Mekinian, Arsene |
| Contributors: |
CHU Lille; Inserm; Institut Pasteur de Lille; Université de Lille; Facteurs de risque et déterminants moléculaires des maladies liées au vieillissement (RID-AGE) - U1167; Centre de Recherche Saint-Antoine CRSA; CHU Saint-Antoine AP-HP; Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases; Service de médecine interne CHU Caen; Institut Pierre Louis d'Epidémiologie et de Santé Publique iPLESP; Hôpital de la Timone CHU - APHM TIMONE; Service de médecine interne Lille; Institut Arnault Tzanck; Hôpital Foch Suresnes; Service de Médecine Interne CHU Rouen; Hôpital-Clinique Claude-Bernard Metz; CHRU Brest - Service d'Hématologie CHU-Brest-Hemato; Service d'Hématologie CHRU Nancy; Institut Gustave Roussy IGR; Hôpital Lariboisière-Fernand-Widal APHP; Hôpital Cochin AP-HP; Centre de néphrologie et transplantation rénale Hôpital de la Conception - APHM; Centre Hospitalier Universitaire de Nantes = Nantes University Hospital CHU Nantes; Groupe hospitalier nord Bourgoin-Jallieu; Centre hospitalier Bretagne Atlantique (Morbihan) CHBA; Centre Hospitalier Régional Universitaire CHU Lille CHRU Lille; Centre Hospitalier Universitaire d'Angers CHU Angers; Centre Hospitalier d’Hyères; Service d'Hématologie Biologique Béclère; AP-HP - Hôpital Antoine Béclère Clamart; Service de Néphrologie - Hémodialyses CHU Clermont-Ferrand; CHU Montpellier; CH de Saint-Malo Broussais; Hôpital Universitaire Carémeau Nîmes CHU Nîmes; Service de Médecine Interne CHU Clermont-Ferrand; Service d’Hématologie Biologique CHU Clermont-Ferrand; Centre Hospitalier Universitaire Strasbourg CHU Strasbourg; Hôpital Ambroise Paré AP-HP; Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen CLCC Henri Becquerel; Hopital Saint-Louis AP-HP AP-HP; Groupe Hospitalier Intercommunal Le Raincy-Montfermeil; Service de Cytogénétique Médicale CHU Clermont-Ferrand |
| Publication Year: |
2024 |
| Collection: |
LillOA (Lille Open Archive - Université de Lille) |
| Subject Terms: |
Myelodysplastic syndrome; Outcome; Vasculitis |
| Description: |
Our objective was to evaluate characteristics, treatment and outcome of vasculitis associated with myelodysplastic syndrome (MDS) and chronic myelomonicytic leukemia (CMML) PATIENTS AND METHODS: Retrospective descriptive analysis of MDS/CMML-related vasculitis and comparison with MDS/CMML patients without dysimmune features. Seventy patients with vasculitis and MDS/CMML were included, with median age of 71.5 [21-90] years and male/female ratio of 2.3. Vasculitis was diagnosed prior to MDS/CMML in 31 patients (44%), and after in 20 patients. In comparison with MDS/CMML without autoimmune/inflammatory features, vasculitis with MDS/MPN showed no difference in MDS/CMML subtypes distribution nor International Prognostic Scoring System and CMML-specific prognostic (IPSS/CPSS) scores. Vasculitis subtypes included Giant cell arteritis in 24 patients (34%), Behçet's-like syndrome in 11 patients (20%) and polyarteritis nodosa in 6 patients (9%). Glucocorticoids (GCs) were used as first-line therapy for MDS/CMML vasculitis in 64/70 patients (91%) and 41 (59%) received combined immunosuppressive therapies during the follow-up. After a median follow-up of 33.2 months [1-162], 31 patients (44%) achieved sustained remission. At least one relapse occurred in 43 patients (61%). Relapse rates were higher in patients treated with conventional Disease Modifying Anti-Rheumatic Drug (DMARDs) (odds ratio 4.86 [95% CI 1.38 - 17.10]), but did not differ for biologics (odds ratio 0.59 [95% CI 0.11-3.20]) and azacytidine (odds ratio 1.44 [95% CI 0.21-9.76]) than under glucocorticoids. Overall survival in MDS/CMML vasculitis was not significantly different from MDS/CMML patients without autoimmune/inflammatory features (p = 0.5), but acute leukemia progression rates were decreased (log rank |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
32fb29c6-52cc-4361-a4cd-50c852423ca5; Seminars in Arthritis and Rheumatism; Semin. Arthritis Rheum.; http://hdl.handle.net/20.500.12210/40748 |
| Availability: |
https://hdl.handle.net/20.500.12210/40748 |
| Rights: |
info:eu-repo/semantics/closedAccess |
| Accession Number: |
edsbas.2D9CAEB3 |
| Database: |
BASE |