Background/Purpose: To describe characteristics, treatment and outcome of patients with systemic inflammatory or autoimmune diseases (SAID) and myelodysplastic syndrome (MDS).
Methods: For this retrospective study, a questionnaire was sent to SNFMI, CRI and GFM to report patients with SAID and MDS, concomitantly or successively. SAID were classified according to usual diagnostic criteria. Exclusion criteria included 1) MDS diagnosed > 12 months after immunosuppressive treatment for SAID 2) infectious, drug-related or neoplasm-related SAID. MDS characteristics, outcome, survival were compared between patients with MDS associated SAID and a cohort of 665 MDS without SAID diagnosed at Avicenne’s university hospital between 2003 and 2013.
Results:
123 patients with both MDS and SAID (mean age, 70±13 years; 41 females and 82 males, baseline characteristics in table 1) were included. SAID was systemic vasculitis in 39 cases (32%), connective tissue diseases in 31 cases (25%), inflammatory arthritis in 28 cases (23%), neutrophilic disorder in 12 cases (10%) and unclassified in 13 cases (11%). Complete diagnostic SAID criteria were fulfilled in 66% of cases, remained incomplete in 21% and SAID was unclassified in the remaining patients. The diagnosis of SAID and MDS was concomitant in 38 (31%) cases, diagnosis of SAID preceded MDS in 46 (37%) cases and was made after MDS in 39 (32%) cases, with the time between the diagnoses of the 2 diseases being 8.6±52 months. Apart from significant association between Chronic myelomonocytic leukemia (CMML) and systemic vasculitis (p=0.0024), no correlation was seen between specific types of SAID and of MDS. A response to SAID first line treatment (mainly steroids), was observed in 83% of the 118 treated cases, including 80% for steroids alone. A second-line treatment was required for steroid dependence or relapse in 48% of the patients. Among treated patients who received biologic targeted treatments at any time (n=27), overall response of SAID (partial or complete) was noted in 9/20 (45%) patients. Among 16 patients treated by azacytidine for their MDS, SAID remission was seen at 3 months in 75% of the cases, with a significant decrease of acute-phase reactants and steroid amounts required. At last follow-up, 37 patients (67%) with stable MDS had remission of SAID, and among patients with MDS progression, 23 patients (56%) also had active SAID (p=0.2).
Conclusion:
The spectrum of SAID associated to MDS is variable, many cases remain difficult to classify. Presence of SAID has no impact on the overall survival of MDS patients. Azacitidine can improve SAID in 75% of the patients. Because of frequent steroid dependence and relapse of SAID, better therapeutic strategies with biological targeted drugs are required, while larger use of MDS specific dugs like azacitidine must be assessed prospectively.
Table 1. Baseline characteristics of patients with MDS-associated to SAID and MDS without SAID.
|
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MDS with SAID N=123
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MDS without SAID N=665
|
|
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Age (years)
|
70±13 |
73±11* |
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Female/Male
|
41/82 (50%) |
291/374 (78%)* |
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||
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Karyotype Favorable Intermediate Poor
|
62 (75%) 8 (10%) 13 (16%) |
386 (69%) 111 (20%)* 64 (11%)* |
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||
|
Bone Marrow blasts (%)
|
6.5±9 |
4±5* |
|
||
|
IPSS
|
0.9±0.9 |
0.8±0.9 |
|
||
|
IPSS low Intermediate-1 Intermediate-2 Poor
|
18 (23%) 39 (49%) 15 (19%) 7 (9%) |
190 (34%)* 181 (33%)* 107 (19%) 76 (14%) |
|
||
|
RCUD
|
11 (9%) |
73 (11%) |
|
||
|
RARS
|
1 (1%) |
57 (9%)*
|
|
||
|
RAEB-1
|
18 (15%) |
130 (20%) |
|
||
|
RAEB- 2
|
10 (8%) |
116 (17%)* |
|
||
|
CMML 1 /2
|
19(16%) / 5 (4%) |
96 (14%) / 7 (1%) |
|
||
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5q syndrome
|
6 (5%) |
25 (4%) |
|
||
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RCMD
|
31 (26%) |
136 (20%) |
|
||
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MDS-U
|
11 (9%) |
22 (3%) |
|
||
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Progression to Acute Leukemia
|
26 (22%) |
83 (21%) |
|||
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Survival (medians, months)
|
72 [59-105] |
75 [48-300] |
|
||
*p<0.05
Disclosure:
A. Mekinian,
None;
E. Grignano,
None;
T. Braun,
None;
O. Decaux,
None;
E. Liozon,
None;
N. Costedoat-Chalumeau,
None;
J. E. Kahn,
None;
M. Hamidou,
None;
G. Falgarone,
None;
O. Lortholary,
None;
S. Park,
None;
Z. Amoura,
None;
A. Mathian,
None;
B. Gombert,
None;
C. Rose,
None;
X. Puechal,
None;
D. Launay,
None;
G. Denis,
None;
B. Lioger,
None;
A. L. Buchdaul,
None;
S. georgin Lavialle,
None;
F. Montestruc,
None;
M. Omouri,
None;
J. Rossignol,
None;
J. M. Ziza,
None;
P. Cathebras,
None;
S. Madaule,
None;
B. de Wazières,
None;
N. Morel,
None;
S. Trouillet,
None;
L. Raffray,
None;
Y. Schoindre,
None;
E. Toussirot,
None;
J. C. Piette,
None;
C. Gardin,
None;
L. Ades,
None;
P. Fenaux,
None;
O. Fain,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/systemic-inflammatory-and-autoimmune-manifestations-associated-with-myelodysplastic-syndrome-a-french-multicenter-retrospective-study/