Long-term outcomes and chronological remaining normal lung volumes in patients with interstitial lung disease with anti-aminoacyl tRNA synthetase antibodies and anti-melanoma differentiation-associated gene 5 antibodies

Yamasaki Yoshioki, Iida Harunobu, Ando Takayasu, Goto Yutaka, Suzuki Shotaro, Kawasaki Tatsuya, Ishizaki Yoshiki, Sakurai Keiichi, Kiyokawa Tomofumi, Mizushima Machiko, Takakuwa Yukiko, Tonooka Kumiko, Yamazaki Kazuko, Imamura Mitsuru, Ooka Seido, Nagafuchi Hiroko, Kawahata Kimito, Matsuoka Shin

doi:10.14961/cra.34.152

Objective: To evaluate long-term outcomes and multi-slice computed tomography（MSCT）-measured chronological remaining normal lung volume in patients with interstitial lung disease（ILD）having the anti-aminoacyl tRNA synthetase（ARS）or the anti-melanoma differentiation-associated gene 5（MDA5）antibodies.Methods: We retrospectively investigated 51 ILD patients with anti-ARS（n = 38）or anti-MDA5（n = 13）between January 2014 and May 2019. Patients were classified as having polymyositis/dermatomyositis（PM/DM）, clinically amyopathic DM（CADM）, or interstitial pneumonia with autoimmune features. The MSCT-based chronological remaining lung volumes were measured.Results: A total of 29%, 45%, and 5%, and 0%, 31%, and 54% of patients with anti-ARS and anti-MDA5 had PM/DM/CADM, respectively. The MSCT-measured mean（SD）remaining lung volumes at baseline were 3100（720）mL and 2796（887）mL in the anti-ARS and anti-MDA5 groups, respectively（P = 0.226）. Survival was more favorable in patients with anti-ARS than in those with anti-MDA5（93% vs. 77% at 4-year, P = 0.022）. Remission was achieved in 94% and 85% of patients with anti-ARS and anti-MDA5, respectively, who received induction therapy. The remaining lung volumes did not significantly increase over time [mean（SD）, 21（9）months] in the anti-ARS group（P = 0.867）but improved in the anti-MDA5 group（P = 0.030）. The mean（SD）annual percent change was 104（18）% vs. 119（15）% in anti-ARS-positive vs. anti-MDA5-positive patients（P = 0.055）.Conclusion: Despite the favorable survival of anti-ARS-positive patients, ILD improvement was minimal. Long-term therapeutic strategies for ILD in this subset of patients are warranted.

Long-term outcomes and chronological remaining normal lung volumes in patients with interstitial lung disease with anti-aminoacyl tRNA synthetase antibodies and anti-melanoma differentiation-associated gene 5 antibodies

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