Assessment of disability in idiopathic inflammatory myopathy: a call for linearity

Rheumatology (Oxford). 2022 Aug 3;61(8):3420-3426. doi: 10.1093/rheumatology/keab906.

Abstract

Objectives: To evaluate the clinimetric properties of the Academic Medical Centre Disability Score (ALDS) in patients with idiopathic inflammatory myopathy (IIM).

Methods: We used prospectively collected data of IIM patients who completed a phase-2 study with first-line IVIG monotherapy. The ALDS is a patient-reported questionnaire which contains 25 items relevant for disability in myositis. ALDS and all core set measures (CSMs) for myositis [including HAQ-Disability Index (HAQ-DI)] were evaluated at baseline and 9 weeks follow-up. In addition, the 2016 ACR/EULAR myositis response criteria outcome called Total Improvement Score (TIS) was evaluated at 9 weeks. We examined floor/ceiling effects, reliability and construct validity of the ALDS. To examine known-group validity, ALDS change scores over time were compared with TIS and physician impression of clinical response.

Results: Nineteen patients with IIM [median age 59 years, 12 (63%) female] were enrolled. At baseline, ALDS showed a median score of 65.4 (IQR 58.2-73.5), good Cronbach's alpha (α = 0.84) and a small ceiling effect (11%). Construct validity was confirmed by moderate to strong correlations between ALDS and HAQ-DI [rs = -0.57 (baseline); -0.86 (follow-up)]. ALDS change score correlated with TIS (rs = 0.70), discriminated between responders and non-responders (TIS ≥ 40; P = 0.001), between groups based on physician impression of clinical response (P = 0.03), and detected deterioration.

Conclusion: The ALDS showed promising clinimetric properties and detected relevant changes in disability in patients with myositis. These results warrant further investigations.

Keywords: ALDS; clinimetric evaluation; disability score; idiopathic inflammatory myopathies; myositis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Disability Evaluation*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Myositis* / diagnosis
  • Myositis* / drug therapy
  • Reproducibility of Results
  • Surveys and Questionnaires