Abstract
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.
Highlights
Risk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-interstitial lung diseases (ILD))
GAP and Interstitial lung disease – gender (ILD-GAP) are applicable for evaluating the risk of death of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) in a similar manner as in those with IPF
As far as we are aware, neither composite physiologic indexes (CPI) nor GAP/ILD-GAP have been previously investigated in patients with RAILD, if one excludes the subjects in the original ILDGAP publication, which did include some RA-ILD
Summary
Risk 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 subsequently developed GAP model combines gender (G), age (A) and two lung physiology variables (P), i.e. forced vital capacity (FVC) and diffusion capacity to carbon monoxide (DLCO), into a multidimensional index and staging system with three stages (I-III) proposing 1-year mortality of 6, 16 and 39% [8]. This GAP model has been utilized in the prognosis of other chronic ILDs in addition to IPF. As far as we are aware, neither CPI nor GAP/ILD-GAP have been previously investigated in patients with RAILD, if one excludes the subjects in the original ILDGAP publication, which did include some RA-ILD patients in their CTD-ILD/idiopathic nonspecific interstitial pneumonia (iNSIP) group of 326 patients
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