Physiological Profile and Limitations in Exercise in Idiopathic Pulmonary Fibrosis.
OBJECTIVE
This study aimed to describe the physiological profile and limiting factors during exercise among patients with idiopathic pulmonary fibrosis.


METHODS
A descriptive study involving 34 patients with idiopathic pulmonary fibrosis (22 men) aged 68 ± 8 years was conducted. All patients completed a pulmonary function test, cardiopulmonary exercise test, Doppler echocardiography, 6-minute walk test, and modified Medical Research Council dyspnea evaluation.


RESULTS
Approximately 38% of patients (range, 15%-71%) presented with coexisting comorbidities including pulmonary hypertension and emphysema. Modified Medical Research Council grades 0-2 and 3-4 were assigned to 68% and 32% of patients, respectively. Median values for forced vital capacity and diffusion capacity for carbon monoxide percent (%) predicted were 68 (95% CI, 63-76) and 51 (95% CI, 46-55), respectively. Left ventricular systolic function was normal. Aerobic capacity ((Equation is included in full-text article.)O2peak = 13.4 mL/kg/min [95% CI, 12.6-14.9]; 62% predicted [95% CI, 56-67]) was moderately reduced with the presence of abnormalities in pulmonary gas exchange and desaturation, circulatory impairments, inefficient ventilation, and skeletal muscle dysfunction. Functional capacity was normal (6-minute walk test distance = 505 m [95% CI, 435-522]; 99% predicted [95% CI, 91-108]).


CONCLUSIONS
The physiological profile demonstrated the presence of comorbidities in approximately 38% of patients with idiopathic pulmonary fibrosis and a moderate level of dyspnea. Resting cardiopulmonary function showed moderate pulmonary restriction and severe impairments in diffusion capacity with normal left ventricular systolic function. Multifactorial limitations for a moderately diminished aerobic capacity were revealed during the cardiopulmonary exercise test, although functional capacity was normal. These results emphasize the significance of a meticulous evaluation, including the cardiopulmonary exercise test for an accurate exercise tolerance evaluation, to aid in the diagnosis and prognosis and for developing effective targeted treatments.
Pulmonary Institute, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel (Drs Vainshelboim, Fox, Ollech, and Kramer); Research Center in Physical Activity, Health and Leisure (CIAFEL), Faculty of Sport, University of Porto, Porto, Portugal (Drs Vainshelboim and Oliveira); Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel (Drs Fox and Kramer); and Pulmonary Division, Lady Davis Carmel Medical Center, Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel (Dr Adir).
Baruch
B
Vainshelboim
Jose
J
Oliveira
Benjamin Daniel
BD
Fox
Yochai
Y
Adir
Jacob Eliezer
JE
Ollech
Mordechai Reuven
MR
Kramer
D000368:Aged; D015308:Anaerobic Threshold; D004417:Dyspnea / Q000209:etiology; D015150:Echocardiography, Doppler; D015444:Exercise / Q000502:physiology*; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011653:Pulmonary Diffusing Capacity; D011656:Pulmonary Emphysema / Q000150:complications / Q000503:physiopathology; D012146:Rest / Q000502:physiology; D013599:Systole; D016277:Ventricular Function, Left; D014797:Vital Capacity; D000070857:Walk Test