Patient Perspectives in OMERACT Provide an Anchor for Future Metric Development and Improved Approaches to Healthcare Delivery in Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD)

Abstract

Objective The impact and natural history of connective tissue disease related interstitial lung disease (CTD-ILD) are poorly understood; and have not been previously described from the patient’s perspective. This investigation sought insight into CTD-ILD from the patients’ perspective to add to our knowledge of CTD-ILD, identify disease-specific areas of unmet need and gather potentially meaningful information towards development of disease-specific patient-reported outcome measures (PROMs). Methods A mixed methods design incorporating patient focus groups (FGs) querying disease progression and life impact followed by questionnaires with items of importance generated by >250 ILD specialists were implemented among CTD-ILD patients with rheumatoid arthritis, idiopathic inflammatory myopathies, systemic sclerosis, and other CTD subtypes. FG data were analyzed through inductive analysis with five independent analysts, including a patient research partner. Questionnaires were analyzed through Fisher’s Exact tests and hierarchal cluster analysis. Results Six multicenter FGs included 45 patients. Biophysiologic themes were cough and dyspnea, both pervasively impacting health related quality of life (HRQoL). Language indicating dyspnea was unexpected, unique and contextual. Psycho-social themes were Living with Uncertainty, Struggle over Self-Identity, and Self-Efficacy - with education and clinician communication strongly emphasised. All questionnaire items were rated ‘moderately’ to ‘extremely’ important with 10 items of highest importance identified by cluster analysis. Conclusion Patients with CTD-ILD informed our understanding of symptoms and impact on HRQoL. Cough and dyspnea are central to the CTD-ILD experience. Initial FGs have provided disease-specific content, context and language essential for reliable PROM development with questionnaires adding value in recognition of patients’ concerns.

Connective Tissue Disease-associated Interstitial Lung Diseases (CTD-ILD) — Report from OMERACT CTD-ILD Working Group

“…Living with uncertainty was a theme where patients described confusion regarding their diagnosis and prognosis; discussions emphasized the need for improved communication to aid patients’ perceptions and understanding of their health/health condition. This manuscript has been submitted for publication (9) (unpublished observation).…”

Performance of the St George’s Respiratory Questionnaire in patients with connective tissue disease‐associated interstitial lung disease

“…Interstitial lung disease (ILD) is a common pulmonary manifestation associated with connective tissue disease (CTD), resulting in significant morbidity and mortality . Patients with CTD‐ILD often experience impaired health ‐related quality of life (HRQoL) …”

Incorporating the patient's perspective in outcomes research

“…Mittoo et al [47] conducted a mixed methods study to evaluate the impact of interstitial lung disease (ILD) in patients with connective tissue diseases (CTDs). Forty-five patients with CTD-ILD reported that cough and dyspnea have significant impacts on patients’ ability to complete activities of daily living.…”