Associations between Patient-reported Outcomes and Death or Lung Transplant in Idiopathic Pulmonary Fibrosis. Data from the Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry

Case, Amy Hajari; Hellkamp, Anne S.; Neely, Megan L.; Bender, Shaun; Dilling, Daniel F.; Gulati, Mridu; Hotchkin, David; Huie, Tristan J.; Lancaster, Lisa; Snyder, Laurie D.; Conoscenti, Craig; Palmer, Scott M.

doi:10.1513/annalsats.201906-437oc

Cited by 23 publications

(18 citation statements)

References 35 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Regarding idiopathic pulmonary brosis (IPF), most prevalent original disease in the present study, mMRC dyspnea [19] and SGRQ [20,21] also predicted mortality, although well-known multi-dimensional severity scoring for IPF such as CPI (composite physiologic index) [22] or GAP (gender, age and physiology) [23] didn't include these measures.…”

Section: Discussionmentioning

confidence: 68%

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

Ikeda

Oga

Chen‐Yoshikawa

et al. 2021

Preprint

View full text Add to dashboard Cite

Background Waitlist mortality due to donor shortage for lung transplantation is a serious problem worldwide. Currently, the selection of recipients is mainly based on registration order in Japan. However, scientific evidence for risk stratification for waitlist mortality is needed in future. We hypothesized that patient-reported dyspnea and health would predict mortality in patients waitlisted for lung transplantation. Methods Using data on 203 patients who were registered as candidates for lung transplantation from deceased donors, we analyzed factors related to waitlist mortality. Dyspnea was evaluated by the modified Medical Research Council (mMRC) dyspnea scale and health status was measured with the St. George’s Respiratory Questionnaire (SGRQ). Results Among 197 patients who met inclusion criteria, the main underlying disease was interstitial pneumonia (IP) in 99 patients. During the median follow-up period of 572 days, 72 patients on the waitlist died and 96 received lung transplantation (69 from deceased donor). Univariable competing risk analyses revealed that both mMRC dyspnea and SGRQ Total were significantly associated with waitlist mortality (p = 0.003 and p < 0.001). Multivariable competing risk analyses revealed that the mMRC and SGRQ were associated with waitlist mortality, among age, IP, arterial carbon dioxide pressure, and forced vital capacity, which were all significant factors in univariable analyses. Conclusions Both mMRC dyspnea and SGRQ were significantly associated with waitlist mortality regardless of patients’ background, underlying disease, and pulmonary function. Patient-reported dyspnea and health should be measured not only from the perspective of multi-dimensional analysis including subjective perceptions, but also as risk stratification for waitlist mortality.

show abstract

Section: Discussionmentioning

confidence: 68%

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

Ikeda

Oga

Chen‐Yoshikawa

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Subsequent studies reported that this approach can predict future outcomes, such as mortality in patients with COPD (predictive property) [ 7 , 8 ]. Regarding idiopathic pulmonary fibrosis (IPF), the most prevalent original disease in the present study, mMRC dyspnea [ 19 ] and SGRQ [ 20 , 21 ] also predicted mortality. Notably, well-established multi-dimensional severity scoring for IPF, such as the composite physiologic index [ 22 ] or GAP (gender, age and physiology) [ 23 ], did not include these measures.…”

Section: Discussionmentioning

confidence: 78%

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

et al. 2021

View full text Add to dashboard Cite

Background Waitlist mortality due to donor shortage for lung transplantation is a serious problem worldwide. Currently, the selection of recipients in Japan is mainly based on the registration order. Hence, scientific evidence for risk stratification regarding waitlist mortality is urgently needed. We hypothesized that patient-reported dyspnea and health would predict mortality in patients waitlisted for lung transplantation. Methods We analyzed factors related to waitlist mortality using data of 203 patients who were registered as candidates for lung transplantation from deceased donors. Dyspnea was evaluated using the modified Medical Research Council (mMRC) dyspnea scale, and the health status was determined with St. George’s Respiratory Questionnaire (SGRQ). Results Among 197 patients who met the inclusion criteria, the main underlying disease was interstitial lung disease (99 patients). During the median follow-up period of 572 days, 72 patients died and 96 received lung transplantation (69 from deceased donors). Univariable competing risk analyses revealed that both mMRC dyspnea and SGRQ Total score were significantly associated with waitlist mortality (p = 0.003 and p < 0.001, respectively) as well as age, interstitial lung disease, arterial partial pressure of carbon dioxide, and forced vital capacity. Multivariable competing risk analyses revealed that the mMRC and SGRQ score were associated with waitlist mortality in addition to age and interstitial lung disease. Conclusions Both mMRC dyspnea and SGRQ score were significantly associated with waitlist mortality, in addition to other clinical variables such as patients’ background, underlying disease, and pulmonary function. Patient-reported dyspnea and health may be measured through multi-dimensional analysis (including subjective perceptions) and for risk stratification regarding waitlist mortality.

show abstract

“…The relationship between HRQL and mortality in IPF has been studied using SGRQ, but results are divergent. In two studies, baseline HRQL was shown to be significantly associated with mortality [ 31 , 32 ], whereas two other studies did not find a significant association in multivariate analyses [ 33 , 34 ]. Kreuter et al found a significant association between HRQL at last available follow-up and mortality, but no association with change in HRQL from baseline [ 35 ].…”

Section: Discussionmentioning

confidence: 99%

Validation of a derived version of the IPF-specific Saint George’s Respiratory Questionnaire

et al. 2021

View full text Add to dashboard Cite

Background Health-related quality of life (HRQL) is impaired in patients with idiopathic pulmonary fibrosis (IPF). HRQL is often measured using the St. George’s Respiratory Questionnaire (SGRQ) despite the development of an IPF-specific version (SGRQ-I). Using data from a real-world cohort of patients with IPF, we aimed to transform SGRQ into a derived version of SGRQ-I, SGRQ-Ider, to examine the cross-sectional and longitudinal validity of SGRQ-Ider and to compare SGRQ-Ider to SGRQ-I. Methods Based on results from SGRQ, SGRQ-Ider was derived applying the algorithm used to develop SGRQ-I. Of the 50 items in SGRQ, 34 items were retained in SGRQ-Ider. Response options for seven items were collapsed and minor adjustments were made to the weights of two items after correspondence with the developers of SGRQ-I. Cross-sectional validation, responsiveness and minimal clinically important difference (MCID) were assessed by comparison to other HRQL instruments, pulmonary function tests and 6-min walk test performed at baseline, 6 and 12 months. Furthermore, the association between SGRQ-Ider scores and mortality was examined. Results A total of 150 IPF patients participated and 124 completed follow-up at 12 months. SGRQ-Ider performed comparably to SGRQ-I with a high concurrent validity, good test–retest reliability and high known-groups validity. SGRQ-Ider was responsive to change in HRQL and physiological anchors. MCID of SGRQ-Ider for improvement and deterioration was 3.5 and 5.7, respectively. SGRQ-Ider scores were associated with mortality in both univariate (HR 1.82, 95% CI 1.42–2.34 per 20-point increase) and multivariate analyses (HR 1.57, 95% CI 1.20–2.05 per 20-point increase). Conclusions The SGRQ-Ider is a valid, reliable and responsive HRQL instrument in patients with IPF and has psychometric properties comparable to SGRQ-I. Thus, SGRQ results can reliably be transformed into the SGRQ-Ider. The MCID estimates were calculated for improvement and deterioration separately. Increasing SGRQ-Ider score was associated with increased mortality.

show abstract

Associations between Patient-reported Outcomes and Death or Lung Transplant in Idiopathic Pulmonary Fibrosis. Data from the Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry

Cited by 23 publications

References 35 publications

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

Patient-reported dyspnea and health predict waitlist mortality in patients waiting for lung transplantation in Japan

Validation of a derived version of the IPF-specific Saint George’s Respiratory Questionnaire

Contact Info

Product

Resources

About