COPD has a profound impact on daily life, yet remains underdiagnosed and undertreated. We set out to develop a brief, reliable, self-scored questionnaire to identify individuals likely to have COPD. COPD-PS TM development began with a list of concepts identified for inclusion using expert opinion from a clinician working group comprised of pulmonologists (n = 5) and primary care clinicians (n = 5). A national survey of 697 patients was conducted at 12 practitioner sites. Logistic regression identified items discriminating between patients with and without fixed airflow obstruction (AO, postbronchodilator FEV 1 /FVC < 70%). ROC analyses evaluated screening accuracy, compared scoring options, and assessed concurrent validity. Convergent and discriminant validity were assessed via COPD-PS and SF-12v2 score correlations. For known-groups validation, COPD-PS differences between clinical groups were tested. Test-retest reliability was evaluated in a 20% sample. Of 697 patients surveyed, 295 patients met expert review criteria for spirometry performance; 38% of these (n = 113) had results indicating AO. Five items positively predicted AO ( p < 0.0001): breathlessness, productive cough, activity limitation, smoking history, and age. COPD-PS scores accurately classified AO status (area under ROC curve = 0.81) and reliable (r = 0.91 MD, MSc, San Jacinto Methodist Hospital, Baytown, TX; Mary Issac, MD, MPH, St. Joseph's Community Care Hospital, Tampa, FL; Fernando J. Martinez, MD, MS, University of Michigan, Ann Arbor, MI; Phillip Menashe, MD, Yakima Chest Clinic, Yakima, WA; Anna W. Parkman, RRT, PhD, Ohio Dominican University, Columbus, OH; Michael A. Russoniello, MD, St. Clares Hospital, Denville, NJ; Frederic D. Seifer, MD, Takoma Adventist Hospital, Greeneville, TN. Correspondence to: Fernando J. Martinez, MD, MS, Division of Pulmonary and Critical Care Medicine, University of Michigan Health System, Ann Arbor, MI 48109 USA; email: fmartine@umich.edu COPD: Journal of Chronic Obstructive Pulmonary DiseaseApril 2008 85 AO scored significantly higher (6.8, SD = 1.9; p < 0.0001) than patients without AO (4.0, SD = 2.3). Higher scores were associated with more severe AO, bronchodilator use, and overnight hospitalization for breathing problems. With the prevalence of COPD in the studied cohort, a score on the COPD-PS of greater than five was associated with a positive predictive value of 56.8% and negative predictive value of 86.4%. The COPD-PS accurately classified physicianreported COPD (AUC = 0.89). The COPD-PS is a brief, accurate questionnaire that can identify individuals likely to have COPD.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with great variability in disease severity and rate of progression. The need for a reliable, sensitive, and objective biomarker to track disease progression and response to therapy remains a great challenge in IPF clinical trials. Over the past decade, quantitative computed tomography (QCT) has emerged as an area of intensive research to address this need. We have gathered a group of pulmonologists, radiologists and scientists with expertise in this area to define the current status and future promise of this imaging technique in the evaluation and management of IPF. In this Pulmonary Perspective, we review the development and validation of six computer-based QCT methods and offer insight into the optimal use of an imaging-based biomarker as a tool for prognostication, prediction of response to therapy, and potential surrogate endpoint in future therapeutic trials.

Assessment of health-related quality of life (HRQL) is particularly important in patients with progressive and incurable diseases such as idiopathic pulmonary fibrosis (IPF). The St George’s Respiratory Questionnaire (SGRQ) has frequently been used to measure HRQL in patients with IPF, but it was developed for patients with obstructive lung diseases. The aim of this review was to examine published data on the psychometric performance of the SGRQ in patients with IPF. A comprehensive search was conducted to identify studies reporting data on the internal consistency, construct validity, test-retest reliability, and interpretability of the SGRQ in patients with IPF, published up to August 2013. In total, data from 30 papers were reviewed. Internal consistency was moderate for the SGRQ symptoms score and excellent for the SGRQ activity, impact and total scores. Validity of the SGRQ symptoms, activity, impact and total scores was supported by moderate to strong correlations with other patient-reported outcome measures and with a measure of exercise capacity. Most correlations were moderately strong between SGRQ activity or total scores and forced or static vital capacity, the most commonly used marker of IPF severity. There was evidence that changes in SGRQ domain and total scores could detect within-subject improvement in health status, and differentiate groups of patients whose health status had improved, declined or remained unchanged. Although the SGRQ was not developed specifically for use with patients with IPF, on balance, its psychometric properties are adequate and suggest that it may be a useful measure of HRQL in this patient population. However, several questions remain unaddressed, and further research is needed to confirm the SGRQ’s utility in IPF.Electronic supplementary materialThe online version of this article (doi:10.1186/s12955-014-0124-1) contains supplementary material, which is available to authorized users.

Background Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). Conclusions In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/ . Trial registration ClinicalTrials.gov number: NCT01915511 . Electronic supplementary material The online version of this article (10.1186/s12931-019-1043-9) contains supplementary material, which is available to authorized users.

IntroductionWe evaluated the psychometric properties of the St George’s Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis (IPF) using data from the two INPULSIS trials.MethodsData from 1061 patients treated with nintedanib or placebo were pooled. Internal consistency, test–retest reliability, construct validity, known-groups validity, responsiveness and responder thresholds were examined.ResultsCronbach’s α was 0.93 for SGRQ total score and >0.75 for domain scores. In patients with stable disease based on change in forced vital capacity (FVC) ≤5% predicted or ‘no change’ on Patient’s Global Impression of Change, intraclass correlation coefficients for the SGRQ total score were 0.72 or 0.76, respectively. Moderate to strong correlations were observed between SGRQ total and domain scores and the Cough and Sputum Assessment Questionnaire cough domains (−0.34 to −0.65), University of California San Diego Shortness of Breath Questionnaire (0.56 to 0.83) and EuroQol 5-Dimensional Quality of Life Questionnaire Visual Analogue Scale (−0.41 to −0.55); correlations with FVC % predicted were weak (−0.24 to −0.30). Longitudinal correlations between changes in SGRQ total score and these patient-reported outcomes over 52 weeks were moderate. Changes in SGRQ total, impact and activity scores were sensitive to detecting improvement or deterioration in FVC >10% predicted at week 52. Collectively, distribution-based and anchor-based approaches suggested using a change of 4–5 points in SGRQ total score as a starting point for responder analyses.ConclusionsThe psychometric properties of the SGRQ support its use as a measure of health-related quality of life in patients with IPF.