Comparison of Outcomes Between Protocolized Tacrolimus-Based and Non-Protocolized Ciclosporin-Based Triple-Combination Therapy in Interstitial Lung Disease With Anti-MDA5-Positive Dermatomyositis: A Single-Center Retrospective Cohort Study.

Aim: We retrospectively compared the outcomes of protocolized tacrolimus-based and non-protocolized ciclosporin-based triple-combination therapies in consecutive patients with newly diagnosed interstitial lung disease (ILD) and antimelanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis (DM).

Methods: Clinical data from consecutive adult patients with newly diagnosed anti-MDA5-positive DM-associated ILD in our hospital from 2013 to 2022 were analyzed. Recent cases received protocolized therapy with high-dose glucocorticoids (GCs), tacrolimus, and intravenous cyclophosphamide (IVCY). Earlier cases received non-protocolized ciclosporin-based triple-combination therapy. The observation period was 12 months. The outcomes included a composite of death or requirement for long-term domiciliary oxygen therapy (LTOT), mortality, GC and IVCY doses, and cytomegalovirus reactivation rates within 12 months.

Results: Protocolized therapy with tacrolimus (n = 14) and non-protocolized therapy with ciclosporin (n = 10) groups had similar baseline characteristics. No deaths or LTOT were observed in the protocolized therapy with the tacrolimus group, whereas four patients in the non-protocolized therapy with ciclosporin group experienced these outcomes (difference, 40 percentage points; 95% CI, 10 to 70; p = 0.020). The 12-month mortality rates were not significantly different between the two groups (p = 0.16). The protocolized therapy with the tacrolimus group had lower GC dosages at 6 months, more total cycles and cumulative doses of IVCY, and lower cytomegalovirus reactivation rates than the non-protocolized therapy with the ciclosporin group (p < 0.05).

Conclusions: Protocolized tacrolimus-based triple-combination therapy demonstrated better outcomes than non-protocolized ciclosporin-based triple-combination therapy in patients with anti-MDA5-positive DM-associated ILD. Reduced GC doses and more intensive IVCY use also presumably contributed to better outcomes in the protocolized therapy with the tacrolimus group.