There is very little data available regarding health-related quality of life (HRQoL) in rare diseases, including idiopathic inflammatory myopathy (IIM). To look at this issue further, abstract 207 (Friday Poster session) sought to investigate the magnitude of impairment in HRQoL, measured using the Medical Outcomes Trust Short Form-36 (SF-36), and the clinical correlates of physical HRQoL in early IIM.
Leclair and her colleagues at McGill University studied a group of patients inthe Inflammatory Myopathy Study cohort. Thirty-eight IIM patients were enrolled, the majority women with a mean disease duration of 1.3 +/- 1.5 years. Subtypes of IIM included dermatomyositis (43%), clinically amyopathic dermatomyositis (19%), connective tissue disease-associated myositis (24%), polymyositis (11%) and cancer-associated myositis (3%). Subjects underwent standardized clinical histories, medical examinations, and self-administered questionnaires at time of entry. Multiple linear regression was used to assess the relationship between selected demographic and clinical variables and the SF-36 Physical Component Summary (PCS) score.
Interstitial lung disease (ILD) was seen in 62%, inflammatory arthritis in 35%, and dysphagia in 24%. In all eight SF-36 domains, considerable impairment was noted on physical functioning as well as the mental component summary score. In multiple linear regression analysis, there was a strong association between ILD and the SF-36 PCS.
This study showed that, in IIM patients, HRQol is very impaired. This is definitely seen in the physical domains. When ILD was present in early IIM, there was a clinically meaningful reduction in physical health status.
The take away message from this is that, if ILD is present in IIM patients, optimal treatment and optimization of care could potentially improve HRQoL.