Abstract Rationale Comprehensive symptom assessment is lacking in myositis, as patient-reported outcomes (PROs) are rarely collected systematically. This study evaluates the feasibility of PRO collection and examines the associations between respiratory symptoms (dyspnea, cough) and quality of life (QoL) in myositis without interstitial lung disease (ILD), myositis-associated ILD, and idiopathic pulmonary fibrosis (IPF). Methods Adult patients with pulmonologist- or rheumatologist-confirmed diagnosis of myositis with or without ILD and patients with IPF were invited to complete electronic PROs through REDCap during the week of a pulmonology or rheumatology visit, including: FACIT Dyspnea Severity and Functional Limitations 10-item Short Forms (T-scores, higher is worse), Leicester Cough Questionnaire (LCQ) (3-21, higher is better), PROMIS Fatigue Short Form (SF) 7a, PROMIS Pain Interference SF 6a, and PROMIS Physical Function SF 8b (T-scores, higher indicates more of the construct). Response rates and survey completion time were recorded. Questionnaire scores were compared across disease groups and by FACIT Dyspnea Severity or LCQ scores above or below the median using ANOVA and t-tests. Results 53 patients (15 myositis only, 26 myositis-ILD, 12 IPF) participated (response rate 53/201, 26%). Patients with myositis only or myositis-ILD were younger (mean SD age: 55 18 and 60 13 years) and more likely to be female (93% and 73%) compared to patients with IPF (age 74 7 years, 8% female) (p 0. 01 for both). Disease duration was a median 6 (IQR 3-11) and 5 (2-8) years for myositis and ILD, respectively. Completion time for all 5 PROs was a median 8. 5 (IQR 5. 0-12. 6) minutes and did not differ by diagnosis (p = 0. 06). Mean FACIT Dyspnea Severity T-scores did not differ across groups (myositis only: 45 12, myositis-ILD: 43 15, IPF: 46 12; p = 0. 81). Mean LCQ Total scores were worse in IPF (myositis only: 20 2, myositis-ILD: 19 3, IPF: 17 3; p = 0. 03). PROMIS Fatigue, Pain Interference, and Physical Function T-scores did not differ across groups (p 0. 05 for all). Greater dyspnea associated with worse QoL in all diagnosis groups (Figure 1). Greater cough associated with worse QoL in myositis-ILD and IPF (p 0. 05), but not in myositis only. Conclusions Collection of multi-domain PROs in myositis care is feasible. Dyspnea serves as an indicator of poor QoL and functional impairment across all disease groups, while cough is more common in ILD. Longitudinal studies are essential to determine whether improvement in one domain leads to secondary improvement in dyspnea. Understanding this directionality is crucial for designing targeted, multi-disciplinary interventions. This abstract is funded by: NIH Rheumatology Training Grant T32-AR076951-05; NHLBI 5R01HL169392-02
Romich et al. (Fri,) studied this question.