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
Specialty: Pulmonology
ICD-10: ,