Background and aims Interstitial lung disease (ILD) can be associated with the idiopathic inflammatory myopathies (IIM). Rapidly progressive interstitial lung disease (RP-ILD) has been recognised in Asian cohorts and has a high mortality. This study aims to describe a cohort of RP-ILD in Singapore, and identify factors associated with RP-ILD.

Methods This is a retrospective study of IIM-ILD patients in the Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital. Patient symptoms, clinical and serologic features, mortality were compared.

Results There were 68 IIM-ILD patients from 2003–12, 63 cases were analysed. RP-ILD was identified in 14 (22%). The RP-ILD group was more likely to be male (42.9% versus 24.5%), were more likely to present with fever (50% vs 14.3%, p=0.01), cough (71.4% vs 34.7%, p=0.03), and dyspnea at rest (21.4% vs 2%, p=0.03). A larger proportion of patients with RP-ILD had amyopathic dermatomyositis (ADM) (35.7% vs 20.4%). None of the patients with RP-ILD had anti-Jo1 (0% vs 34%, p=0.01). All RP-ILD cases deteriorated, with 80% requiring mechanical ventilation. The mortality was 100% in the RP-ILD group (vs 16% in non RP-ILD group, p=0.00). Median time from diagnosis to death was 26 days.

Conclusions We identified ADM and absence of anti Jo-1 as strong associations for RP-ILD. Mortality was 100% in this RP-ILD cohort. Clinicians should have heightened awareness of this phenotype, early management at specialised respiratory care units, aggressive combination immunosuppressive therapy may be key to mortality reduction.