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A pilot treatment study of patients with anti-aminoacyl-tRNA synthetase antibody–positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) shows therapy with glucocorticoids and calcineurin inhibitors (CNI) to be highly effective.
A cohort of 32 consecutive ARS-PM/DM-ILD patients were studied over time to assess the efficacy of first-line prednisolone (PSL) plus CNI therapy was compared with that of PSL alone using propensity score–matched analysis. Progression free survival (PFS) was was defined as no deterioration in PM/DM-ILD (including recurrence).
Patients were randomized to with PSL + CNI (n=20) and PSL (n=12).
For all patients the 2-year PFS was 69% and the 5-year survival rate was 96.9%. The 2-year PFS rate was significantly higher in the matched PSL + CNI group than in the matched PSL group.
The 5-year survival rates of both the matched PSL + CNI and PSL groups were favorable.
First-line PSL + CNI therapy in ARS-PM/DM-ILD patients yieleded better PFS compared with PSL monotherapy, yet there was no significant difference regarding longterm survival.