Efficacy of calcineurin inhibitors in interstitial lung disease of patients with antisynthetase syndrome: a systematic literature review and single-arm prevalence meta-analysis.

Abstract

Objectives: Interstitial lung disease (ILD) of antisynthetase syndrome (ASyS) is one of the most common and significant clinical features of this disease, and it is associated with a worse prognosis. An increasing number of studies on the efficacy of calcineurin inhibitors (CNI) in this field have been published. This study aimed to assemble these data to better determine the efficacy of CNIs in ASyS-ILD.

Methods: Cochrane Reviews, MEDLINE, Ovid, Scopus, and Web of Science were searched for studies that used CNIs (cyclosporine A or tacrolimus) in combination with steroids to treat ILD associated with ASyS. Meta-analyses of the efficacy of CNIs were performed, with proportions used as effect sizes for overall efficacy, efficacy in acute/subacute ILD, efficacy in chronic ILD, and ILD relapses under treatment.

Results: A total of twelve studies were eligible. Meta-analyses demonstrated that CNIs use resulted in improvement and stable disease in 88.4 % (95 % Confidence Interval-CI: 78.2-95.7) of patients with ASyS-ILD (overall), in 91.5 % (95 % CI: 65.5-99.9) of patients with acute/subacute ASyS-ILD, and in 84.9 % (95% CI: 64.0-97.6) of patients with chronic ASyS-ILD. However, 56.4 % (95% CI: 47.9-65.2) of ASyS-ILD patients relapsed under CNI treatment.

Conclusions: CNIs, in combination with glucocorticoids, can be effective for treating acute/subacute and chronic ILD in patients with ASyS. However, relapses of ILD can frequently occur under this treatment modality. Future studies are needed to identify patients who have a higher risk of relapse.