THU0388 Differences in clinical courses and serum markers of interstitial lung disease associated with anti-aminoacyl-transfer rna synthetase antibody and anti-melanoma differentiation-associated gene 5 antibody-positive polymyositis/dermatomyositis
K. Akashi,Yoko Nose,Taiichiro Shirai,Y. Fujikawa,T. Nagamoto,T. Okano,S. Takahashi,S. Sendo,Akira Onishi,Jun Saegusa,Akio Morinobu +10 more
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MDA5-ILD patients should be monitored for both rapidly progressive disease and relapsing disease, including the changes in serum ILD markers, including a normal SP-D level, which is a feature of MDA 5-ILD.Abstract:
Background Polymyositis/dermatomyositis (PM/DM) is a chronic autoimmune disease that is often complicated by interstitial lung disease (ILD). Anti-aminoacyl-transfer RNA synthetase antibody (ARS-Ab) and anti-melanoma differentiation-associated gene 5 antibody (MDA5-Ab) are highly detected in PM/DM with ILD. It was reported that ARS-Ab-positive-ILD (ARS-ILD) is often recurrent1, and patients with MDA5-Ab-positive-ILD (MDA5-ILD) develop fatal rapidly progressive ILD2. Objectives To evaluate the differences in clinical courses between ARS-ILD and MDA5-ILD, including the changes in serum ILD markers. Methods We retrospectively investigated 25 patients with ARS-ILD and 26 patients with MDA5-ILD who received induction therapy between April 2002 and September 2017 at Kobe University Hospital. The survival rate and relapse-free survival rate were analysed with Kaplan-Meier estimation and the log-rank test. The differences in serum ILD markers between patients with ARS-ILD and MDA5-ILD were evaluated with the Student’s t-test. Results Disease subtypes at diagnosis with PM/DM-associated ILD were as follows: Eleven ARS-ILD and no MDA5-ILD patients had PM, 10 ARS-ILD and 5 MDA5-ILD patients had DM, and 4 ARS and 21 MDA5 patients had amyopathic DM. The survival rate for MDA5-ILD was significantly lower than that for ARS-ILD (p Conclusions MDA5-ILD patients should monitored for both rapidly progressive disease and relapsing disease. A normal SP-D level is a feature of MDA5-ILD. References [1] Nakazawa M, Kaneko Y, Takeuchi T. Risk factors for the recurrence of interstitial lung disease in patients with polymyositis and dermatomyositis: a retrospective cohort study. Clin Rheumatol2017. Epub ahead of print. [2] Hozumi H, Fujisawa T, Nakashima R, et al. Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease. Respir Med2016;121:91–9. Acknowledgements none. Disclosure of Interest None declaredread more
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