Extensive interstitial lung disease in inflammatory myopathy is a strong predictor of mortality

The objective of this study was to identify clinical predictors of mortality in a prospective cohort of subjects with IIM.Subjects were from the Canadian Inflammatory Myopathy Study group, a multicenter, prospective cohort of incident IIM. Clinical data, lung function were obtained per study protocol. Computed tomography scans (CT) of the chest done for clinical indications were reviewed by a respirologist for presence of ILD. Total lung involvement was estimated to the nearest 5%. Kaplan-Meier was used to identify survival differences between groups with and without ILD. Cox proportional hazard model identified predictors of mortality.There were 65 subjects included in this study. Mean age was 53 (SD 16.3). Mean FVC was 90.6% (SD 22%) and mean DLCO was 83% (SD 28%) predicted. ILD was present in 32 (49%) subjects. Survival was significantly lower in subjects with >20% ILD on CT (log rank p<0.01; Figure 1). The strongest predictor of mortality was presence of ILD involving >20% of the lung (HR 14.88, 95% CI 1.52 – 145, p=0.02). This remained true after adjusting for age.ILD is common in IIM. Having >20% ILD on CT was associated with significantly increased mortality. This simple staging system provides important prognostic information for these patients.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Diffuse Parenchymal Lung Disease Source Type: research