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Multicenter Study
. 2020 Oct;50(5):879-884.
doi: 10.1016/j.semarthrit.2020.07.002. Epub 2020 Jul 11.

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

Anne Laure Roupie  1 , Alexis Guedon  2 , Benjamin Terrier  3 , Constance Lahuna  1 , Vincent Jachiet  1 , Alexis Regent  3 , Hubert de Boysson  4 , Fabrice Carrat  5 , Julie Seguier  6 , Louis Terriou  7 , Mathilde Versini  8 , Viviane Queyrel  8 , Matthieu Groh  9 , Ygal Benhamou  10 , Francois Maurier  11 , Emmanuel Ledoult  12 , Lenaig Le Clech  13 , Maud D'Aveni  14 , Julien Rossignol  15 , Joris Galland  16 , Lise Willems  17 , Noemie Jourde Chiche  18 , Pierre Peterlin  19 , Marielle Roux-Sauvat  20 , Anne Parcelier  21 , Matthieu Wemeau  22 , Marc Lambert  7 , Cristina Belizna  23 , Xavier Puechal  3 , Laure Swiader  6 , Rolande Cohen-Valensi  24 , Valérie Noc  25 , Emmanuel Dao  25 , Sylvain Thepot  26 , Grégoire Martin de Frémont  2 , Aline Tanguy-Schmidt  26 , Anne Marfaing Koka  27 , Guillaume Bussone  28 , Carole Philipponnet  29 , Amadou Konate  30 , Guilhem Cavaille  17 , Philippe Guilpain  30 , Jean-Sébastien Allain  31 , Jonathan Broner  32 , Eric Solary  15 , Marc Ruivard  33 , Benoit de Renzis  34 , Sélim Corm  35 , Nadia Baati  36 , Nicolas Schleinitz  6 , Matthieu Ponsoye  37 , Aspasia Stamatoullas-Bastard  38 , Lionel Ades  39 , Azeddine Dellal  40 , Andrei Tchirkov  41 , Achille Aouba  30 , Pierre Fenaux  41 , Olivier Fain  1 , Arsène Mekinian  42 ; On behalf MINHEMON (French Network of dysimmune disorders associated with hemopathies) and SNFMI.
Affiliations
Multicenter Study

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

Anne Laure Roupie et al. Semin Arthritis Rheum. 2020 Oct.

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.

Keywords: Myelodysplastic syndrome; Outcome; Vasculitis.

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