ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1343

Efficacy of Biological-Targeted Treatments in MDS-Related Systemic Autoimmune Diseases: Multicenter Retrospective Study of 28 Patients

Guillaume Dervin1, Arsène Mékinian2, Jean-Emmanuel Kahn3, louis Terriou4, Eric Liozon5, Eric Grignano6, odile beyne Rauzy7, Pascal Godmer8, Julien Rossignol9, Geraldine Falgarone10, Laurence Bouillet11, Achille Aouba12, Philippe Guilpain13, David Launay14, jonathan Broner15, jerome gillard16, lionel ades17, clemence salvado18, thierry cardon19, Jean-Charles Piette20, Pierre Fenaux21 and Olivier Fain2, 1Medecine Interne Hopital Saint antoine, Paris, France, 2Service de médecine interne. Hôpital Saint-Antoine., Paris, France, 3Internal Medicine, Foch Hospital, Suresnes, France, 4médecine interne CHRU lille, Lille, France, 5Departement of Internal Medicine, Limoges University Hospital, Limoges, France, 6Internal Medicine, DHUi2B Saint Antoine Hospital, paris, France, 7Medecine interne CHU Purpan, toulouse, France, 8CH Vannes, Vannes, France, 9haematology, paris, France, 10Medecine Interne Hopital Avicenne, Bobigny, France, 11CHU, Grenoble, France, 12Medecine Interne Hopital Necker, Paris, France, 13Medecine interne CHU Montpellier, Montpellier, France, 14Service de Médecine Interne, Centre National de Référence des Maladies Systémiques Rares, Hôpital Claude Huriez, CHRU Lille, Lille, France, 15Medecine Interne CHRU Montpellier, Montpellier, France, 16Medecine Interne CHRU Lons le Saunier, Lons, France, 17Service Hématologie Hopital d'Avicennes, Bobigny, France, 18Service d'hématologie CHU Mondor, Creteil, France, 19Medecine interne CHU Lille, lille, France, 20Internal Medicine, Pitié-Salpêtrière University Hospital, Paris, France, 21Hematologie Hopital Avicenne, Bobigny, France

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: ,

Session Information

Date: Monday, November 14, 2016

Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: This study analyze the safety and efficiency of biologics (TNF-α antagonists, tocilizumab, rituximab and IL-1 inhibitors) in patients with autoimmune systemic diseases (SAIDs) associated to myelodysplastic syndrome (MDS).

Methods: In a French multicenter retrospective study, 28 patients with both MDS and SAID treated with at least one biological-targeted treatment were analyzed. Clinical, biological and overall treatment response was considered as complete (no more symptoms), partial (at least 50% improvement) or non-response. In patients with several lines of treatment, data were analyzed before and at the end of each line and pooled to compare overall response between steroids, DMARDs and biologics

Results: 28 patients with median age 66 [19-85] years, 11% of females were included. MDS most frequent subtypes were CMML (18%), AREB-1 (32%), CRDM (32%), 5q-(7%) with median marrow blasts 2% (0-10), normal karyotype in 12/24 (50%) cases and IPSS 0.5 (0-1). The associated SAIDs were arthritis (undifferentiated and rheumatoid arthritis in 3 (10%) cases each), Behcet’s disease in 2 (7%) cases, cryoglobulinemic vasculitis in 2 (7%) cases, giant cell arteritis in 1 (4%), relapsing polychondritis in 8 (30%) cases, Sweet’s disease in 3 (10%) cases and others (18%). During the follow-up of 3 (1.3-4.5) years, 112 lines of treatments were used, consisting in steroids alone (23%), DMARDs (25%), TNF-α antagonists (15%), anakinra (11%), rituximab (10%) and tocilizumab (7%). The median number of lines was 3 (1-9) and more than 5 lines were used in 13 (46%) of patients. Specific MDS treatment was used for SAIDs in 10 patients (9%) (azacytidine). Considering all 112 lines, overall response (complete and partial) occurred in 54% cases. Comparing the treatments, overall response was noted more frequently under steroids (76%) and rituximab (54%), than DMARDs (45%) and TNF-α antagonists, tocilizumab and anakinra (31%)(p<0.05). Azacytidine allowed 71% response in steroid-dependent SAIDs. Steroid dependence occured in 25 cases with mean steroid dose 27 mg/day (10-120). During the follow-up, 20 (71%) patients presented at least one severe infection. Table 1

N=112

DMARDs

N=29 (26%)

Biologics

N=36 (32%)

 

Rituximab

N=11 (10%)

Steroids

N=26 (24%)

Azacytidine/BMT

N=10 (9%)

Treatments

Steroids

21 (95%)

36 (95%)

11(91%)

25 (100%)

10 (100%)

Prednisone (mg/day) before/at the end

25 (18-38)

25 (15-28)

25 (20-30)

22.5 (10-30)

20 (15-30)

18 (9-24)

60 (40-60)

25 (20-40)

25 (18-28)

20 (5-23)

Type of immunosuppressive agents

MTX 7 (24%)

Anakinra

14 (39%)

–

–

–

 

CYC

4 (13%)

TNF-a antagonists

15 (42%)

–

–

–

 

AZA

4 (13%)

Tocilizumab

7 (19%)

–

–

–

 

MMF

5 (18%)

–

–

–

–

 

CICLO

5 (18%)

–

–

–

–

HCQ

4(14%)

Treatment duration (months)

9 (4-21)

2.5 (1-7)

6 (4-32)

9 (2-12)

8 (3-22)

C-reactive protein level (mg/l) before/at the end

53 (39-140)

18 (3-200)

60 (22-90) /

60 (26-99)

18 (5-50)

30 (7-50)

90 (45-117)

32 (4-90)

53 (36-79)

9 (3-20)

SAID response

SAIDs partial response

1/20 (5%)

8/30(25%)

4/11 (36%)

1/23 (4%)

5/7 (71%)

SAIDs complete response

8/20(40%)

2/30 (6%)

2/11 (18%)

18/23 (78%)

0

SAIDs non response

11/20 (55%)

21/30 (69%)

5/10 (46%)

4/23 (18%)

2/7 (29%)

Conclusion: This nationwide study demonstrates the efficiency of steroids for SAID associated to MDS, the frequency of steroid dependence and poor response to biologics, except rituximab. The efficiency of azacytidine for MDS-related SAIDs should be considered in patients with inefficacy of other strategies and steroid dependence.

Disclosure: G. Dervin, None; A. Mékinian, None; J. E. Kahn, None; L. Terriou, None; E. Liozon, None; E. Grignano, None; O. beyne Rauzy, None; P. Godmer, None; J. Rossignol, None; G. Falgarone, None; L. Bouillet, None; A. Aouba, None; P. Guilpain, None; D. Launay, None; J. Broner, None; J. gillard, None; L. ades, None; C. salvado, None; T. cardon, None; J. C. Piette, None; P. Fenaux, None; O. Fain, None.

To cite this abstract in AMA style:

Dervin G, Mékinian A, Kahn JE, Terriou L, Liozon E, Grignano E, beyne Rauzy O, Godmer P, Rossignol J, Falgarone G, Bouillet L, Aouba A, Guilpain P, Launay D, Broner J, gillard J, ades L, salvado C, cardon T, Piette JC, Fenaux P, Fain O. Efficacy of Biological-Targeted Treatments in MDS-Related Systemic Autoimmune Diseases: Multicenter Retrospective Study of 28 Patients [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/efficacy-of-biological-targeted-treatments-in-mds-related-systemic-autoimmune-diseases-multicenter-retrospective-study-of-28-patients/. Accessed .

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