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

被引：30

作者：

Roupie, Anne Laure ^{[1
,2
,3
]}

Guedon, Alexis ^{[3
]}

Terrier, Benjamin ^{[4
]}

Lahuna, Constance ^{[1
,2
,3
]}

Jachiet, Vincent ^{[1
,2
,3
]}

Regent, Alexis ^{[4
]}

de Boysson, Hubert ^{[5
]}

Carrat, Fabrice ^{[6
]}

Seguier, Julie ^{[7
]}

Terriou, Louis ^{[8
]}

Versini, Mathilde ^{[9
]}

Queyrel, Viviane ^{[9
]}

Groh, Matthieu ^{[10
]}

Benhamou, Ygal ^{[11
]}

Maurier, Francois ^{[12
]}

Ledoult, Emmanuel ^{[13
]}

Le Clech, Lenaig ^{[14
]}

D'Aveni, Maud ^{[15
]}

Rossignol, Julien ^{[16
]}

Galland, Joris ^{[17
]}

Willems, Lise ^{[18
]}

Chiche, Noemie Jourde ^{[19
]}

Peterlin, Pierre ^{[20
]}

Roux-Sauvat, Marielle ^{[21
]}

Parcelier, Anne ^{[22
]}

Wemeau, Matthieu ^{[23
]}

Lambert, Marc ^{[8
]}

Belizna, Cristina ^{[24
]}

Puechal, Xavier ^{[4
]}

Swiader, Laure ^{[7
]}

Cohen-Valensi, Rolande ^{[25
]}

Noc, Valerie ^{[26
]}

Dao, Emmanuel ^{[26
]}

Thepot, Sylvain ^{[27
]}

de Fremont, Gregoire Martin ^{[3
]}

Tanguy-Schmidt, Aline ^{[27
]}

Koka, Anne Marfaing ^{[28
]}

Bussone, Guillaume ^{[29
]}

Philipponnet, Carole ^{[30
]}

Konate, Amadou ^{[31
]}

Cavaille, Guilhem ^{[18
]}

Guilpain, Philippe ^{[31
]}

Allain, Jean-Sebastien ^{[32
,36
]}

Broner, Jonathan ^{[33
]}

Solary, Eric ^{[16
]}

Ruivard, Marc ^{[34
]}

de Renzis, Benoit ^{[35
]}

Corm, Selim ^{[36
]}

Baati, Nadia ^{[37
]}

Schleinitz, Nicolas ^{[7
]}

机构：

[1] Hop St Antoine, AP HP, Inflammat Immunopathol Biotherapy Dept DHU I2B, Dept Internal Med, 184 Rue Faubourg St Antoine, F-75012 Paris, France

[2] Sorbonne Univ, Paris, France

[3] Sorbonne Univ, INSERM U938, Ctr Rech St Antoine CRSA, Paris, France

[4] Univ Paris, Hop Cochin, Assistance Publ Hop AP HP, Dept Internal Med,Natl Reference Ctr Rare & Syst, Paris, France

[5] CHU Caen, Dept Internal Med, Ave Cote Nacre, F-14033 Caen, France

[6] Sorbonne Univ, Hop St Antoine, AP HP, INSERM,Inst Pierre Louis Epidemiol & Sante Publ, F-75012 Paris, France

[7] Hop La Timone, AP HM, Dept Internal Med, Marseille, France

[8] CHU Lille, Dept Internal Med, Lille, France

[9] Inst Arnault Tzanck, St Laurent Du Var, France

[10] Foch Hosp, Natl Referral Ctr Hypereosinophil Syndromes CEREO, Dept Internal Med, Suresnes, France

[11] CHU Rouen, Dept Internal Med, Rouen, France

[12] Hop Prives Metz, Dept Internal Med, Metz, France

[13] CHU Lille, Dept Internal Med & Clin Immunol, Lille, France

[14] CH Cornouaille, Dept Hematol, Quimper, France

[15] CHU Nancy, Dept Hematol, Nancy, France

[16] Gustave Roussy Canc Ctr, Dept Hematol, F-94805 Villejuif, France

[17] Univ Paris, Hop Lariboisiere, AP HP, Dept Internal Med, Paris, France

[18] Hop Cochin, AP HP, Dept Hematol, F-75014 Paris, France

[19] Hop Conception, AP HM, Ctr Nephrol & Transplantat Renale, Dept Nephrol, Marseille, France

[20] CHU Nantes, Dept Hematol, Nantes, France

[21] CH Pierre Oudot, Dept Internal Med, Bourgoin Jallieu, France

[22] Ctr Hosp Bretagne Atlantique, Dept Hematol, Vannes, France

[23] CHU Lille, Dept Hematol, Lille, France

[24] CHU Angers, UMR CNRS 6015 INSERM, Dept Vasc Med, Angers, France

[25] CH Martigues Hop Rayettes, Dept Internal Med, Martigues, France

[26] CH Hyeres, Dept Nephrol, Hyeres, France

[27] CHU Angers, Dept Hematol, Angers, France

[28] Hop Antoine Beclere, Dept Hematol, Clamart, France

[29] Hop Antoine Beclere, Dept Internal Med, Clamart, France

[30] CHU Clermont Ferrand, Dept Nephrol, Clermont Ferrand, France

[31] CHU Montpellier, Dept Internal Med, Montpellier, France

[32] CH St Malo, Pole Cardio Vasc & Metab, St Malo, France

[33] CHU Nimes, Dept Internal Med, Nimes, France

[34] CHU Clermont Ferrand, Dept Internal Med, Clermont Ferrand, France

[35] CHU Clermont Ferrand, Dept Hematol, Clermont Ferrand, France

[36] MedipOle Savoie, Dept Hematol, Charles Les Eaux, France

[37] CH Strasbourg, Dept Hematol, Strasbourg, France

[38] CHU Ambroise Pare, Dept Internal Med, Boulogne Billancourt, France

[39] Ctr Henri Becquerel, Dept Hematol, Rouen, France

[40] Hop St Louis, AP HP, Dept Hematol, 1 Ave Claude Vellefaux, F-75010 Paris, France

[41] Montfermeil Hosp, Dept Rheumatol, F-93370 Montfermeil, France

[42] CHU Clermont Ferrand, Dept Med Cytogenet, Clermont Ferrand, France

来源：

SEMINARS IN ARTHRITIS AND RHEUMATISM | 2020年 / 50卷 / 05期

关键词：

Myelodysplastic syndrome; Vasculitis; Outcome; AUTOIMMUNE; MANIFESTATIONS; AZACITIDINE; SERIES;

D O I：

10.1016/j.semarthrit.2020.07.002

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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%), Behcet'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. (C) 2020 Elsevier Inc. All rights reserved.

引用

页码：879 / 884

页数：6

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