Prognosis Of Adult Idiopathic Inflammatory Myopathy-Associated Interstitial Lung Disease: A Retrospective Study Of 679 Adult Cases

Objectives. Few studies have investigated the prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) across different clinical/serological phenotypes.Methods. We conducted a retrospective analysis of patients diagnosed with IIM between January 2012 and December 2017.Results. Of the 760 IIM cases registered, 679 adult cases were included in this study. ILD was present in 508 cases, and the presence of ILD in the clinically amyopathic DM, DM and PM groups was 92.7, 73.6 and 55.1%, respectively (P < 0.01). The prevalence of ILD in the anti-synthetase antibody (ASA)(+)-IIM group was higher than that in ASA(-)-IIM group (95.2 vs 72.4%, P<0.01); no such difference was found between the anti-histidyl-tRNA synthetase (Jo-1)(+)-IIM and Jo-1(-)ASA(+)-IIM groups (93.0 vs 98.5%, P>0.05). The prevalence of ILD in the melanoma differentiation-associated protein-5 (MDA-5)(+)-IIM group was higher than that in MDA-5(-)-IIM group (97.8 vs 72.1%, P<0.01). Among adults with IIM, men with concurrent ILD, who were older than 50 years, weremost likely to die. No significant difference was found in the all-causemortality rates between DM-ILD and clinically amyopathic DM-ILD groups (33.3 vs 23%, P>0.05), although both were higher than that in PM group (13.2%, P = 0.01 and P<0.05, respectively). No difference was found in the all-cause mortality rates between MDA5- ASA- IM-ILD and MDA5(-)ASA(+)IM-ILD groups (17.2 vs 12.8%, P>0.05), and both were lower than that in MDA5(+)ASA(-)-IM-ILD group (33.7%, P<0.05).Conclusion. The prevalence of ILD in IIM and the prognosis of IIM-ILD patients may vary depending on the statuses of the ASA and MDA-5 antibodies.