Predicting Mortality in Systemic Sclerosis-Associated Interstitial Lung Disease Using Risk Prediction Models Derived From Idiopathic Pulmonary Fibrosis

Ryerson, Christopher J.; O’Connor, Darragh; Dunne, James V.; Schooley, Fran; Hague, Cameron; Murphy, Darra; Leipsic, Jonathon; Wilcox, Pearce

doi:10.1378/chest.15-0003

Cited by 49 publications

(34 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A recent meta-analysis of 6-MWD showed differences in walking distances between groups with or without PH or ILD [69]. For the subgroup of patients with SSc and ILD, the 6-MWD has been included in an algorithm for calculating mortality risk [70]. Similarly, in a meta-analysis of patients with SSc with PH, a shorter 6-MWD was associated with a worse prognosis [53], alongside age, gender, pericardial effusion, increased right atrial pressure, increased PAP mean , and reduced cardiac output.…”

Section: Discussionmentioning

confidence: 99%

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

et al. 2019

View full text Add to dashboard Cite

Background: Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO 2) and ventilation/carbon dioxide (VE/VCO 2)-slope, can predict survival in patients with SSc. Subjects and methods: We retrospectively assessed 210 patients (80.9% female) in 6 centres over 10 years with pulmonary testing and CPET. Survival was analysed with Cox regression analysis (adjusted for age and gender) by age, comorbidity (Charlson-Index), body weight, body-mass index, extensive interstitial lung disease, pulmonary artery pressure (measured by echocardiography and invasively), and haemodynamic, pulmonary and CPET parameters. Results: Five-and ten-year survival of SSc patients was 93.8 and 86.9%, respectively. There was no difference in survival between patients with diffuse (dcSSc) and limited cutaneous manifestation (lcSSc; p = 0.3). Pulmonary and CPET parameters were significantly impaired. Prognosis was worst for patients with pulmonary hypertension (p = 0.007), 6-min walking distance < 413 m (p = 0.003), peakVO 2 < 15.6 mL•kg − 1 •min − 1 , and VE/VCO 2-slope > 35. Age (hazard ratio HR = 1.23; 95% confidence interval CI: 1.14;1.41), VE/VCO 2-slope (HR = 0.9; CI 0.82;0.98), diffusion capacity (Krogh factor, HR = 0.92; CI 0.86;0.98), forced vital capacity (FVC, HR = 0.91; CI 0.86;0.96), and peakVO 2 (HR = 0.87; CI 0.81;0.94) were significantly linked to survival in multivariate analyses (Harrell's C = 0.95). Summary: This is the first large study with SSc patients that demonstrates the prognostic value of peakVO 2 < 15.6 mL•kg − 1 •min − 1 (< 64.5% of predicted peakVO 2) and VE/VCO 2-slope > 35.

show abstract

Section: Discussionmentioning

confidence: 99%

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

et al. 2019

View full text Add to dashboard Cite

show abstract

“…It has since been studied in patients with scleroderma related ILD 24 and other chronic ILDs 25 . While RA-ILD was included in the connective tissue disease (CTD) associated ILD group of a prior publication 25 , their contribution to the study cohort was small (n= 56 out of 281 with CTD-ILD).…”

Section: Discussionmentioning

confidence: 99%

The performance of the GAP model in patients with rheumatoid arthritis associated interstitial lung disease

Morisset

Vittinghoff

Lee

et al. 2017

Respiratory Medicine

View full text Add to dashboard Cite

Background Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is associated with significant morbidity and mortality. Similarities have been observed between patients with idiopathic pulmonary fibrosis (IPF) and the UIP (usual interstitial pneumonia) form of RA-ILD. The GAP (gender, age, physiology) model has been shown to predict mortality in patients with IPF, but its ability to predict mortality in RA-ILD is not known. Methods We identified 309 patients with RA-ILD at 4 academic centers with ongoing longitudinal cohorts of patients with ILD. The primary endpoint was mortality. To handle missing data (n=219 subjects with complete dataset), multiple imputation by iterative chained equations was used. Using the GAP model as a baseline, we assessed improvements in mortality risk prediction achieved by incorporating additional variables. Model discrimination was assessed using the c-index, and calibration was checked by comparing observed and expected incidence of death. Results Patients had a mean age of 65 years and were predominantly female (54%). The mean forced vital capacity (FVC) % predicted was 73 and the mean diffusing capacity for carbon monoxide (DLCO) % predicted was 55. Twenty-four percent of the 236 patients with a high-resolution computed tomography scan available for review had a definite UIP pattern. The original GAP model, including gender, age, FVC%, and DLCO%, had a c-index of 0.746 in our cohort. Calibration of this model was satisfactory at 1, 2 and 3 years. Model discrimination was not meaningfully improved by adding other clinical variables. Conclusion The GAP model that was derived for IPF performs similarly as a mortality risk prediction tool in RA-ILD.

show abstract

“…In another study of IPF patients, the GAP staging was found to be useful for evaluating the IPF severity, revealing statistically significant differences in survival in different GAP stages [12]. On the other hand, the ILD-GAP index displayed poor applicability for the predicted 1-year mortality in systemic sclerosis-associated ILD patients [14]. …”

Section: Discussionmentioning

confidence: 99%

“…Thus, since it is mainly UIP-typed, RA-ILD follows a distinctive disease course from the other CTD-ILDs and it remains unclear which of the prognostic indexes, GAP or ILD-GAP, would be better suited for RA-ILD. There are some reports of the benefits of using the CPI score, GAP and ILD-GAP staging systems in patients with IPF and systemic sclerosis-associated ILD [12–14]. However, as far as we are aware, neither CPI nor GAP/ILD-GAP have been previously investigated in patients with RA-ILD, if one excludes the subjects in the original ILD-GAP publication, which did include some RA-ILD patients in their CTD-ILD/idiopathic nonspecific interstitial pneumonia (iNSIP) group of 326 patients.…”

Section: Introductionmentioning

confidence: 99%

Are risk predicting models useful for estimating survival of patients with rheumatoid arthritis-associated interstitial lung disease?

et al. 2017

View full text Add to dashboard Cite

BackgroundRisk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The purpose of this study was to test the suitability of three prediction models as well as individual lung function and demographic factors for evaluating the prognosis of RA-ILD patients.MethodsClinical and radiological data of 59 RA-ILD patients was re-assessed. GAP (gender, age, physiologic variables) and the modified interstitial lung disease (ILD)-GAP as well as the composite physiologic indexes (CPI) were tested for predicting mortality using the goodness-of-fit test and Cox model. Potential predictors of mortality were also sought from single lung function parameters and clinical characteristics.ResultsThe median survival was 152 and 61 months in GAP / ILD-GAP stages I and II (p = 0.017). Both GAP and ILD-GAP models accurately estimated 1-year, 2-year and 3-year mortality. CPI (p = 0.025), GAP (p = 0.008) and ILD-GAP (p = 0.028) scores, age (p = 0.002), baseline diffusion capacity to carbon monoxide (DLCO) (p = 0.014) and hospitalization due to respiratory reasons (p = 0.039), were significant predictors of mortality in the univariate analysis, whereas forced vital capacity (FVC) was not predictive. CPI score (HR 1.03, p = 0.018) and baseline DLCO (HR 0.97, p = 0.011) remained significant predictors of mortality after adjusting for age.ConclusionsGAP and ILD-GAP are applicable for evaluating the risk of death of patients with RA-ILD in a similar manner as in those with IPF. Baseline DLCO and CPI score also predicted survival.

show abstract

Predicting Mortality in Systemic Sclerosis-Associated Interstitial Lung Disease Using Risk Prediction Models Derived From Idiopathic Pulmonary Fibrosis

Cited by 49 publications

References 22 publications

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

The performance of the GAP model in patients with rheumatoid arthritis associated interstitial lung disease

Are risk predicting models useful for estimating survival of patients with rheumatoid arthritis-associated interstitial lung disease?

Contact Info

Product

Resources

About