Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study

التفاصيل البيبلوغرافية
العنوان:	Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study
المؤلفون:	Mekinian, Arsène, Grignano, Eric, Braun, Thorsten, Decaux, Olivier, Liozon, Eric, Costedoat-Chalumeau, Nathalie, Kahn, Jean-Emmanuel, Hamidou, Mohammed, Park, Sophie, Puéchal, Xavier, Toussirot, Eric, Falgarone, Géraldine, Launay, David, Morel, Nathalie, Trouiller, Sébastien, Mathian, Alexis, Gombert, Bruno, Schoindre, Yoland, Lioger, Bertrand, De Wazieres, Benoit, Amoura, Zahir, Buchdaul, Anne-Laure, Georgin-Lavialle, Sophie, Dion, Jérémie, Madaule, Serge, Raffray, Loïc, Cathebras, Pascal, Piette, Jean Charles, Rose, Christian, Ziza, Jean Marc, Lortholary, Olivier, Montestruc, Francois, Omouri, Mohammed, Denis, Guillaume, Rossignol, Julien, Nimubona, Stanislas, Adès, Lionel, Gardin, Claude, Fenaux, Pierre, Fain, Olivier
بيانات النشر:	Oxford University Press
سنة النشر:	2016
المجموعة:	HighWire Press (Stanford University)
مصطلحات موضوعية:	CLINICAL SCIENCE
الوصف:	Objective. We describe myelodysplastic syndrome (MDS)–associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. Methods. In this study, 123 patients with MDS and SIADs were analysed. Results. Mean age was 70 years (s.d. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutrophilic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis ( P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMML patients without SIADs, MDS/CMML patients with SIADs were younger ( P < 0.01), male ( P = 0.03), less often had refractory anaemia with ring sideroblasts ( P < 0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs ( P = 0.5). Conclusion. The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent.
نوع الوثيقة:	text
وصف الملف:	text/html
اللغة:	English
Relation:	http://rheumatology.oxfordjournals.org/cgi/content/short/55/2/291; http://dx.doi.org/10.1093/rheumatology/kev294
DOI:	10.1093/rheumatology/kev294
الاتاحة:	http://rheumatology.oxfordjournals.org/cgi/content/short/55/2/291 https://doi.org/10.1093/rheumatology/kev294
Rights:	Copyright (C) 2016, British Society for Rheumatology
رقم الانضمام:	edsbas.FFB4085A
قاعدة البيانات:	BASE

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الوصف
DOI:	10.1093/rheumatology/kev294