Patient-reported outcomes to assess dyspnoea in interstitial lung disease and pulmonary hypertension: a systematic literature review of measurement properties

Abstract

Objective:This COnsensus-based Standards for the selection of health measurement INstruments (COSMIN)-based systematic review aims to identify and summarise the quality of measurement properties of dyspnoea-specific patient-reported outcome measures (PROMs) for patients with interstitial lung disease (ILD), pulmonary hypertension (PH) or connective tissue diseases (CTDs).Methods and results:Relevant articles in PubMed and Embase were screened. Based on COSMIN analysis and the Grading of Recommendations, Assessment, Development and Evaluation approach, overall rating and level of evidence were assessed to formulate recommendations. We identified 26 publications on 10 PROMs. For patients with ILD, including CTD-associated ILD, nine PROMs were evaluated, of which the Dyspnea-12 (D12), EXACT-Respiratory Symptoms Idiopathic Pulmonary Fibrosis Breathlessness subscale (ERS-IPF-B), King's Brief Interstitial Lung Disease Health Status Questionnaire breathlessness and activities subscale (KBILD-B) and the University of California San Diego Shortness of Breath Questionnaire (UCSD-SOBQ) had high-quality evidence for sufficient internal consistency, without high-quality evidence of insufficient measurement properties. We reached this same conclusion regarding the D12 for use in patients with PH, including CTD-associated PH. Most PROMs in this systematic review have moderate- or low-quality evidence on construct validity and responsiveness.Conclusion:Four dyspnoea-specific PROMs, D12, ERS-IPF-B, KBILD-B and UCSD-SOBQ, can be recommended for use in patients with ILD, including CTD-associated ILD. Of these four, the D12, despite the limited evidence and the lack of evidence on several important domains, is also suitable for use in patients with PH, including CTD-associated PH.

Changes in the physical and affective dimensions of dyspnoea after a home-based pulmonary rehabilitation in fibrotic idiopathic interstitial pneumonias

“…These tools do not cover the affective dimension of dyspnoea, which may be more associated with the aforementioned comorbidities. Moreover, neither the mMRC scale nor the Borg index are recommended as patient-reported outcome measures [ 37 ], unlike the D-12 questionnaire which was specifically developed and validated in individuals with ILD [ 19 , 20 ]. A previous randomised controlled trial conducted by W adell et al [ 38 ] in patients with COPD considered multidimensional tools for assessing dyspnoea and reported improvements in the affective domain of dyspnoea following an 8-week outpatient PR programme, while the sensory-perceptual domain of dyspnoea was not improved.…”

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

“…Currently, the King's Brief Interstitial Lung Disease (KBILD) questionnaire (KBILD breathlessness and activities subscale) might be considered the best patient-reported outcome measure to evaluate dyspnea, and Dyspnea-12 questionnaire is also suitable for patients with PH; however, evidence is based on a limited number of studies. 87…”

“…Currently, the King's Brief Interstitial Lung Disease (KBILD) questionnaire (KBILD breathlessness and activities subscale) might be considered the best patient-reported outcome measure to evaluate dyspnea, and Dyspnea-12 questionnaire is also suitable for patients with PH; however, evidence is based on a limited number of studies. 87 There is no standardized screening tool for PH in the setting of ILD. 88,89 Suspicion of the disease might be raised by conventional examinations like electrocardiography, usually showing right ventricular hypertrophy and right atrial dilatation, and chest X-ray that may show proximal pulmonary artery and/or right ventricular enlargement.…”

The one-minute sit-to-stand-test performance is associated with health-related quality of life in patients with pulmonary hypertension

“…HRQoL is usually assessed via structured health related patient reported outcome measures. [ 11 ] These measures are based on general well-being across patient groups or specific for certain health conditions. For the evaluation of HRQoL in PH, patients receive a validated PH specific survey, the so-called Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for a thorough assessment of disease specific HRQoL [ 12 ].…”