Fibrose pulmonar idiopática: apresentação clínica, evolução e fatores de prognóstico basais numa coorte portuguesa

Soares, Raquel; Mota, Patrícia Caetano; Melo, Natália; Costa, Diogo; Jesus, José Miguel; Cunha, Rui; Guimarães, Susana; Souto-Moura, Conceição; Morais, António

doi:10.1016/j.rppneu.2012.05.002

Cited by 11 publications

(2 citation statements)

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“…Our results on the characteristics of IPF are broadly consistent with previous studies with respect to age, gender distribution, PFT, survival and smoking history [10, 20, 21]. The patients were relatively uniformly divided into three disease course groups.…”

Section: Discussionsupporting

confidence: 90%

“…Conflicting results on the significance of age as a prognostic factor have been reported in earlier studies. King et al presented that patients less than 50 years old lived longer than their older counterparts whereas in recent studies the age at the time of diagnosis has had no predictive value [21–23]. In contrast, another study revealed that in a multivariate analysis, age and PFT, but not gender, were significant predictors of survival [24].…”

Section: Discussionmentioning

confidence: 99%

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Comparison of disease progression subgroups in idiopathic pulmonary fibrosis

et al. 2019

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BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive interstitial pneumonia with an unpredictable course. The aims of this study were to retrospectively re-evaluate a cohort of patients with IPF according to the 2011 international IPF guidelines and 1) to characterize the subgroups of patients when classified according to their observed survival times and 2) to evaluate whether Composite Physiologic Index (CPI), Gender-Age-Physiology (GAP) Index or clinical variables could predict mortality.MethodsRetrospective data was collected and patients were classified into subgroups according to their observed lifespans. Differences in clinical variables, CPI and GAP stages as well as in comorbidities were investigated between the subgroups. Predictors of mortality were identified by COX proportional hazard analyses.ResultsA total of 132 patients were included in this study. The disease course was rapid (≤ 2 years) in 30.0%, moderate (2–5 years) in 28.0% and slow (≥ 5 years) in 29.0% of the patients. Pulmonary function tests (PFT) and CPI at baseline differentiated significantly between the rapid disease course group and those patients with longer survival times. However, the predictive accuracy of the investigated clinical variables was mainly less than 0.80. The proportions of patients with comorbidities did not differ between the subgroups, but more patients with a rapid disease course were diagnosed with heart failure after the diagnosis of IPF. Most patients with a rapid disease course were categorized in GAP stages I and II, but all patients in GAP stage III had a rapid disease course. The best predictive multivariable model included age, gender and CPI. GAP staging had slightly better accuracy (0.67) than CPI (0.64) in predicting 2-year mortality.ConclusionsAlthough the patients with a rapid disease course could be differentiated at baseline in terms of PFT and CPI, the predictive accuracy of any single clinical variable as well as CPI and GAP remained low. GAP staging was unable to identify the majority of patients with a rapid disease progression. It is challenging to predict disease progression and mortality in IPF even with risk prediction models.

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Section: Discussionsupporting

confidence: 90%