The Clinical Features of Recurrent Interstitial Lung Disease in Dermatomyositis Patients with Anti- Melanoma Differentiation-Associated Gene 5 Antibody

Yuji Hosono¹, Ran Nakashima¹, Kosaku Murakami², Yoshitaka Imura², Naoichiro Yukawa², Hajime Yoshifuji², Koichiro Ohmura² and Tsuneyo Mimori², ¹Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ²Department of Rheumatology and Clinical Immunology, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: cyclophosphamide, cyclosporine, interstitial lung disease and myositis

Session Information

Date: Tuesday, November 10, 2015

Title: Muscle Biology, Myositis and Myopathies Poster II: Autoantibodies and Treatments in Inflammatory Myopathies

Session Type: ACR Poster Session C

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

Background/Purpose: Interstitial lung disease (ILD) is the most common cause of mortality in polymyosytis (PM) and dermatomyositis (DM). It is well known that the DM patients with anti- melanoma differentiation-associated gene 5 (MDA5) antibody (Ab) frequently develop acute progressive ILD and fatal outcome within the first year from the onset. Although survival rate of anti-MDA5-positive patients tends to be sustained after 6 months from the treatment, the characteristics of ILD-recurrent cases are still unclear. Here, we intended to investigate the characteristics of recurrent ILD among anti-MDA5-positive DM-ILD patients.

Methods: Clinical data and serum samples were collected from adult Japanese anti-MDA5-positive DM-ILD patients who visited our department from January 2006 to May 2015. 14 of 20 patients were alive after more than one year from the onset and treated with high dose glucocorticoids (GC) and intravenous cyclophosphamide (IVCY) therapy as the initial treatment. The patients who showed the recurrent symptoms after at least 6 months since the last IVCY therapy were defined as recurrence group. Anti-MDA5 was screened using the immunoprecipitation assay with [³⁵S] methionine-labelled HeLa cells.

Results: 14 patients treated with high dose GC and IVCY therapy as initial treatment survived after more than 6 months. Among these patients, 4 (29%) showed the recurrence. Among them, 3 (75%) were exacerbation of ILD and 1 (25%) was arthritis. The average duration from the last IVCY therapy to recurrence was 42.5±7.9 months. The lymphocyte counts before the initial treatment were significantly lower in recurrence group than in no-recurrence group (556±187 vs 992±118, P<0.05). Moreover, the total dose of IVCY until recurrence was significantly lower in recurrence group (7758±1137 vs 10364±758mg, P<0.05). There were no significant differences in age, sex, duration of diseases, and minimal dose of GC. 3 of 5 (60%) who were treated without calcineurin inhibitors (CNI) showed the exacerbation of ILD, whereas none of 11 who were treated with CNI (P<0.05).

Conclusion: Our study clarified the clinical features of recurrence among anti-MDA5-positive DM-ILD patients. The enough dose of IVCY after the induction of remission combining with CNI as well as high dose GC should be strongly recommended to prevent the recurrence of ILD in anti-MDA5-positive DM-ILD patients.

Disclosure: Y. Hosono, None; R. Nakashima, None; K. Murakami, None; Y. Imura, None; N. Yukawa, None; H. Yoshifuji, None; K. Ohmura, None; T. Mimori, None.

To cite this abstract in AMA style:

Hosono Y, Nakashima R, Murakami K, Imura Y, Yukawa N, Yoshifuji H, Ohmura K, Mimori T. The Clinical Features of Recurrent Interstitial Lung Disease in Dermatomyositis Patients with Anti- Melanoma Differentiation-Associated Gene 5 Antibody [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/the-clinical-features-of-recurrent-interstitial-lung-disease-in-dermatomyositis-patients-with-anti-melanoma-differentiation-associated-gene-5-antibody/. Accessed .

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