TY - JOUR T1 - Efficacy of Glucocorticoids and Calcineurin Inhibitors for Anti-aminoacyl-tRNA Synthetase Antibody–positive Polymyositis/dermatomyositis–associated Interstitial Lung Disease: A Propensity Score–matched Analysis JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.180778 SP - jrheum.180778 AU - Hironao Hozumi AU - Tomoyuki Fujisawa AU - Ran Nakashima AU - Hideki Yasui AU - Yuzo Suzuki AU - Masato Kono AU - Masato Karayama AU - Kazuki Furuhashi AU - Noriyuki Enomoto AU - Naoki Inui AU - Yutaro Nakamura AU - Tsuneyo Mimori AU - Takafumi Suda Y1 - 2019/01/15 UR - http://www.jrheum.org/content/early/2019/01/11/jrheum.180778.abstract N2 - Objective The optimal treatment strategy for anti-aminoacyl-tRNA synthetase antibody–positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) is yet to be established. We aimed to evaluate the efficacy of glucocorticoids and calcineurin inhibitors (CNI) in patients with ARS-PM/DM-ILD. Methods Progression-free survival (PFS) and overall survival rates were retrospectively evaluated in 32 consecutive patients with ARS-PM/DM-ILD. Disease progression was defined as deterioration in PM/DM-ILD (including recurrence). Predictive factors associated with PFS were analyzed by Cox hazards analysis. The efficacy of first-line prednisolone (PSL) plus CNI therapy was compared with that of PSL monotherapy using propensity score–matched analysis. Results Overall, 20 (62.5%) and 12 (37.5%) patients received first-line therapy with PSL + CNI and PSL, respectively. The 2-year PFS and 5-year survival rates in the overall cohort were 68.8% and 96.9%, respectively. On multivariate analysis, arterial oxygen pressure (HR 0.86) and PSL monotherapy (vs PSL + CNI; HR 7.29) showed an independent association with PFS. Baseline characteristics of propensity score-matched PSL + CNI and PSL groups were similar. The 2-year PFS rate was significantly higher in the matched PSL + CNI group than in the matched PSL group. All patients who experienced disease progression during first-line therapy were subsequently treated with second-line therapies. The 5-year survival rates of both the matched PSL + CNI and PSL groups were favorable. Conclusion Propensity score–matched analysis demonstrated that first-line PSL + CNI therapy for patients with ARS-PM/DM-ILD significantly improved the PFS compared with PSL monotherapy, although there was no significant difference regarding longterm survival. ER -