The Early Use of Cyclosporine Is Beneficial for Long-Term Prognosis in Patients of Polymyositis/Dermatomyositis-Associated Interstitial Lung Disease with Anti- Synthetase Antibodies

Yuji Hosono¹, Ran Nakashima², Yoshitaka Imura³, Naoichiro Yukawa², Hajime Yoshifuji², Takaki Nojima⁴, Koichiro Ohmura² and Tsuneyo Mimori², ¹Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, ²Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ³Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ⁴Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: autoantibodies, immunosuppressants, interstitial lung disease and myositis

Session Information

Title: Muscle Biology, Myositis and Myopathies: Myositis Autoantibodies and Disease Phenotype

Session Type: Abstract Submissions (ACR)

Background/Purpose: Interstitial lung disesases (ILD) is the most common cause of mortality in polymyosotis (PM) and dermatomyositis (DM). Cyclosporine had been reported to improve clinical outocome in some patients with PM/DM and associated ILD. However, the efficiencies and longterm prognosis depending on the time of administration are still unclear. Here, we intended to investigate the benefits of early use of cyclosporine in PM/DM-associated ILD with antibodies (Ab) against aminoacyl-tRNA synthetase.

Methods:

Clinical data and serum samples were collected from 144 adult Japanese patients with PM/DM who visited our department from January 2005 to December 2013. Patients who were treated with cyclosporine within 3 months from the initial treatment with high dose glucocorticoids were defined as early use group. Myositis-specific Abs (MSAs) were screened using the RNA immunoprecipitation assay. Kaplan-Meier survival analysis was applied to compare overall mortality rates.

Results:

Sixty eight patients (33 with PM and 35 with DM) were positive for anti- synthetase Ab positive (25 anti-Jo-1, 14 anti-PL-7, 13 anti-EJ, 7 anti-PL-12, 5 anti-OJ, and 4 anti-KS). Among them, 64 patients (94%) had ILD. Among PM/DM associated ILD patients, 11 (17%, 6 with PM and 5 with DM) were cyclosporine early use group. Twenty-one (32%, 7 with PM and 14 with DM) were treated after more than 3 month from initial treatment and 32 (50%, 18 with PM and 14 with DM) were not treated with cyclosporine. The 15-year survival rate of early use group was significantly higher than that of delayed use of cyclosporine and non-combination with cyclosporine groups (P<0.05 by log-rank test). Three of 11 (27%) of early use group did not show any exacerbation of ILD after the treatment, while none of delayed group (P<0.05). There were no significant differences in age, sex, duration of diseases, rates of each anti-synthetase Ab, and requirement of intravenous cyclophosphamide pulse therapy among each group. One of 2 in early use group needed no longer home oxygen therapy　within first year from the initial treatment, whereas none of other groups.

Conclusion:

Our study showed significant survival benefits of cyclosporine use at early stage in PM/DM-associated ILD with anti- synthetase Ab. The introduction of combination therapy with cyclosporine and high dose glucocorticoids at early stage for ILD in PM/DM patients should be strongly recommended.

Disclosure:

Y. Hosono,
None;

R. Nakashima,
None;

Y. Imura,
None;

N. Yukawa,
None;

H. Yoshifuji,
None;

T. Nojima,
None;

K. Ohmura,
None;

T. Mimori,
None.

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