Description

The American Thoracic Society (ATS) and the European Respiratory Society (ERS) issued consensus guidelines for response to therapy in a patient with idiopathic pulmonary fibrosis. These parameters can help guide therapy over time.


 

Parameters:

(1) changes in symptoms (dyspnea, cough)

(2) parenchymal changes in chest images

(3) physiologic changes (spirometry, diffusion, oxygenation)

 

Favorable or improved outcome – at least 2 of the following:

(1) decrease in symptoms, with either an increased level of exertion required before breathless or a decline in the frequency or severity of cough

(2) reduction in parenchymal abnormalities on chest radiograph or high resolution CT

(3) physiologic improvements – at least 2 of the following:

(3a) total lung capacity or vital capacity increase by >= 200 mL or by >= 10%

(3b) single breath DL-CO changes by >= 15 % OR by >= 3 mL per min per mm Hg

(3c) increase in oxygen saturation (>= 4% increase) OR PaO2 (>= 4 mm Hg increase) during a formal cardiopulmonary exercise test

 

Stable and presumed favorable (no disease progression) outcome:

(1) neither favorable nor failure response to therapy

(2) no change in symptoms

(3) no change in parenchymal abnormalities on chest radiograph or high resolution CT

(4) physiologic status – at least 2 of the following:

(3a) total lung capacity or vital capacity change by < 200 mL OR by < 10%

(3b) single breath DL-CO changes by < 15 % AND < 3 mL per min per mm Hg

(3c) no change in oxygen saturation (< 4% change) or PaO2 (< 4 mm Hg change) during a formal cardiopulmonary exercise test

 

Failure to respond to therapy – at least 2 of the following:

(1) increase in symptoms, especially dyspnea and cough

(2) increase in parenchymal abnormalities on chest radiograph or high resolution CT

(3) physiologic deterioration – at least 2 of the following:

(3a) total lung capacity or vital capacity decrease by >= 200 mL or by >= 10%

(3b) single breath DL-CO decreases by >= 15 % OR by >= 3 mL per min per mm Hg

(3c) decrease in oxygen saturation (>= 4% decrease) OR PaO2 (>= 4 mm Hg decrease) during a formal cardiopulmonary exercise test

 


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