Development and Initial Validation Analyses of the Living with Idiopathic Pulmonary Fibrosis Questionnaire — swigris 2020

Idiopathic pulmonary fibrosis is a lung disease that affects around 128,100 persons in the US, with 48,000 new cases being discovered every year. According to 1, idiopathic pulmonary fibrosis is a specific form of chronic, progressive fibrosing interstitial pneumonia that often affects elderly people and has no known cause (ie, aged 50–75 years). As a result of the disorder, lung function and dyspnea (shortness of breath) both deteriorate with time, and the outlook is not promising. Statistics show that idiopathic pulmonary fibrosis has a median survival time of 2.5 to 3.5 years in the United States after diagnosis and that it claims the lives of an estimated 40,000 people annually.

The severity and manifestations of idiopathic pulmonary fibrosis vary from person to person. Some of the symptoms include dyspnea, fatigue, dry coughing, and aching muscles and joints. 4 It is believed that gastroesophageal reflux disease affects nine out of ten patients with idiopathic pulmonary fibrosis. The idiopathic Pulmonary Fibrosis signs and symptoms can have a substantial influence on physical activity, independence, and quality of life.

Idiopathic pulmonary fibrosis can result in cor pulmonale, right-sided heart failure, pulmonary hypertension, lung cancer, respiratory failure, and pulmonary fibrosis.

There is a relationship between acute exacerbations and increased rates of morbidity and mortality.

The average annual direct medical costs for individuals with idiopathic pulmonary fibrosis were $26,378 according to a retrospective analysis of the claims database from 2001 to 2008. These costs were also $12,124 higher than those incurred by control patients who were matched for age and sex.

The number of all-cause outpatient visits and hospital admissions among patients with idiopathic pulmonary fibrosis was double that of the control group over this time.

Occupational therapy for the lungs or oxygen treatment may be helpful for those with idiopathic pulmonary fibrosis. Lung transplantation may be the last option left if all other treatments for some patients with serious illnesses fail.

Idiopathic pulmonary fibrosis patients used to be treated with immunosuppressants and glucocorticoids (like prednisone).

A two-drug combination regimen of prednisone and azathioprine or a three-drug regimen of prednisone, azathioprine, and N-acetylcysteine was used to treat the illness. According to the findings of the PANTHER-IPF trial, which was funded by the National Heart, Lung, and Blood Institute and published in 2012, prednisone, azathioprine, and N-acetylcysteine therapy was demonstrated to significantly increase the risk of mortality and hospitalization when compared to placebo.

According to the US Food and Drug Administration, people with idiopathic pulmonary fibrosis were unable to receive any medication up to October 2014. (FDA).

Strategies that minimize the decline in forced vital capacity may slow the evolution of the illness and improve the outlook for those who are affected.

Swigris JJ, Andrae DA, Churney T, Johnson N, Scholand MB, White ES, Matsui A, Raimundo K, Evans CJ. Development and Initial Validation Analyses of the Living with Idiopathic Pulmonary Fibrosis Questionnaire. Am J Respir Crit Care Med. 2020 Dec 15;202(12):1689-1697. doi: 10.1164/rccm.202002-0415OC. PMID: 32634038; PMCID: PMC7737580.