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


CONCLUSIONS
The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent.
AP-HP, Hôpital Saint Antoine, service de medicine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris.
Arsène
A
Mekinian
AP-HP, Hôpital Saint Antoine, service de medicine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris.
Eric
E
Grignano
Service d'hématologie clinique, Université Paris 13, AP-HP, Avicenne, Bobigny.
Thorsten
T
Braun
Service de médecine interne, Université Rennes 1, Hôpital Universitaire de Rennes, Rennes.
Olivier
O
Decaux
Service de médecine interne, Université Limoges, Hôpital Limoges, Limoges.
Eric
E
Liozon
Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris.
Nathalie
N
Costedoat-Chalumeau
Service de médecine interne, Hôpital Foch, Université Versailles Saint Quentin en Yvelines, Suresnes.
Jean-Emmanuel
JE
Kahn
Service de médecine interne, Hôpital Nantes, Université Nantes, Nantes.
Mohammed
M
Hamidou
Service d'hématologie clinique, Hôpital La Tronche, Grenoble.
Sophie
S
Park
Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris.
Xavier
X
Puéchal
Centre Investigation Clinique Biothérapie CIC-1431 & Service de rhumatologie, CHU Besançon, Besançon.
Eric
E
Toussirot
USPC Université Paris 13, INSERM, APHP; UMR 1125, service de rhumatologie; Hôpital Avicenne, 125 route de Stalingrad, 93009 Bobigny.
Géraldine
G
Falgarone
Service de médecine interne, CHU Lille, Université Lille II, Lille.
David
D
Launay
Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris.
Nathalie
N
Morel
Service de médecine interne, Hôpital Henri Mondor, Aurillac.
Sébastien
S
Trouiller
Service de médecine interne, Hôpital Pitié Salpetrière, Université Paris 6, AP-HP, Paris.
Alexis
A
Mathian
Service de médecine interne et rhumatologie, Groupe hospitalier La Rochelle Ré Aunis, La Rochelle.
Bruno
B
Gombert
Service de médecine interne, Hôpital Foch, Université Versailles Saint Quentin en Yvelines, Suresnes.
Yoland
Y
Schoindre
Service de médecine interne, Hôpital de Tours, Université de Tours, Tours.
Bertrand
B
Lioger
Service de médecine interne et gériatrique, CHU Nîmes, Nîmes.
Benoit
B
De Wazieres
Service de médecine interne, Hôpital Pitié Salpetrière, Université Paris 6, AP-HP, Paris.
Zahir
Z
Amoura
Service de médecine interne, CH Douai, Douai.
Anne-Laure
AL
Buchdaul
Service de médecine interne, Hôpital HEGP, Université Paris 5, AP-HP, Paris.
Sophie
S
Georgin-Lavialle
Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris.
Jérémie
J
Dion
Service de médecine interne, CHG Albi, Albi.
Serge
S
Madaule
Service de médecine interne, CHU de Bordeaux, Bordeaux.
Loïc
L
Raffray
Service de médecine interne, Hôpital Nord CHU Saint Etienne, Saint Etienne.
Pascal
P
Cathebras
Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris.
Jean Charles
JC
Piette
Service d'hématologie clinique, Hôpital Saint-Vincent de Paul, UC Lille, Université Nord de France, Lille.
Christian
C
Rose
Service de rhumatologie, CH Croix Saint Simon.
Jean Marc
JM
Ziza
Service de maladies infectieuses, Hôpital Necker, Université Paris 5, AP-HP, Paris.
Olivier
O
Lortholary
Biostatistics, eXYSTAT SAS, Levallois-Perret.
Francois
F
Montestruc
Service de rhumatologie, CH Romilly/Seine.
Mohammed
M
Omouri
Service de médecine interne, Hôpital de Rochefort, Rochefort.
Guillaume
G
Denis
Service d'hématologie, Hôpital Necker, Université Paris 5, APHP, Paris.
Julien
J
Rossignol
Service d'hématologie clinique, Université Rennes 1, Hôpital Universitaire de Rennes, Rennes and.
Stanislas
S
Nimubona
Service d'hématologie clinique, Hôpital Saint-Louis, AP-HP, Université Paris 7, Paris, France.
Lionel
L
Adès
Service d'hématologie clinique, Université Paris 13, AP-HP, Avicenne, Bobigny.
Claude
C
Gardin
Service d'hématologie clinique, Hôpital Saint-Louis, AP-HP, Université Paris 7, Paris, France.
Pierre
P
Fenaux
AP-HP, Hôpital Saint Antoine, service de medicine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris, olivier.fain@sat.aphp.fr.
Olivier
O
Fain
D000368:Aged; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D015551:Autoimmunity / Q000276:immunology*; D001374:Azacitidine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005602:France; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007249:Inflammation / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D011379:Prognosis; D012189:Retrospective Studies
D000964:Antimetabolites, Antineoplastic; D005938:Glucocorticoids; D001374:Azacitidine
autoimmune disorders; myelodysplastic syndrome; outcome; treatment