Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: French multicenter case-control study.

Abstract

INTRODUCTION:

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.

RESULTS:

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 <0.05).

CONCLUSION:

This study shows no correlation of vasculitis diagnoses with subtypes and severity of MDS/CMML, and no significant impact of vasculitis on overall survival. Whereas conventional DMARDs seem to be less effective, biologics or azacytidine therapy could be considered for even low-risk MDS/CMML vasculitis.

• Authors: Anne Laure Roupie, Alexis Guedon, Benjamin Terrier, Constance Lahuna, Vincent Jachiet, Alexis Regent, Hubert de Boysson, Fabrice Carrat, Julie Seguier, Louis Terriou, Mathilde Versini, Viviane Queyrel, Matthieu Groh, Ygal Benhamou, Francois Maurier, Emmanuel Ledoult, Lenaig Le Clech, Maud D'Aveni, Julien Rossignol, Joris Galland, Lise Willems, Noemie Jourde Chiche, Pierre Peterlin, Marielle Roux-Sauvat, Anne Parcelier, Matthieu Wemeau, Marc Lambert, Cristina Belizna, Xavier Puechal, Laure Swiader, Rolande Cohen-Valensi, Valérie Noc, Emmanuel Dao, Sylvain Thepot, Grégoire Martin de Frémont, Aline Tanguy-Schmidt, Anne Marfaing Koka, Guillaume Bussone, Carole Philipponnet, Amadou Konate, Guilhem Cavaille, Philippe Guilpain, Jean-Sébastien Allain, Jonathan Broner, Eric Solary, Marc Ruivard, Benoit de Renzis, Sélim Corm, Nadia Baati, Nicolas Schleinitz, Matthieu Ponsoye, Aspasia Stamatoullas-Bastard, Lionel Ades, Azeddine Dellal, Andrei Tchirkov, Achille Aouba, Pierre Fenaux, Olivier Fain, Arsène Mekinian, On behalf MINHEMON (French Network of dysimmune disorders associated with hemopathies) and SNFMI.
• Journal: Seminars in arthritis and rheumatism (Semin Arthritis Rheum) Vol. 50 Issue 5 Pg. 879-884 (10 2020) ISSN: 1532-866X [Electronic] United States
• PMID: 32896704 (Publication Type: Journal Article, Multicenter Study)
• Copyright: Copyright © 2020 Elsevier Inc. All rights reserved.

Topics

• Adult
• Aged
• Aged, 80 and over
• Case-Control Studies
• Female
• Giant Cell Arteritis
• Humans
• Leukemia, Myelomonocytic, Chronic (complications, drug therapy, epidemiology)
• Male
• Middle Aged
• Myelodysplastic Syndromes (complications, drug therapy, epidemiology)
• Retrospective Studies
• Young Adult