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321 Rapidly progressive ild in iim – the singaporean experience
  1. TC Tan1,
  2. JWL Tan2,
  3. KP Leong2,
  4. GYL Chan2 and
  5. BYH Thong2
  1. 1Khoo Teck Puat Hospital, General Medicine, Singapore, Singapore
  2. 2Tan Tock Seng Hospital, Rheumatology Allergy and Immunology, Singapore, Singapore

Abstract

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.

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