Update on diagnosis and treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

Untitled

“…Evidence of extrapulmonary manifestations, such as arthralgia, Raynaud phenomenon, dry mouth and eyes, and skin lesions, are essential to the approach for ILDs as these factors can be helpful in establishing the diagnosis of connective tissue diseases (CTDs), which can also present a usual interstitial pneumonia (UIP) pattern. An investigation of the family history of lung disorders is also recommended because CTDs and hereditary diseases are potential etiologies of ILDs (3)(4)(5).…”

“…IPF mainly affects patients in their sixth and seventh decades of life, with a higher prevalence in males and smokers or former smokers, and IPF affects the lungs exclusively (3). Gastroesophageal reflux is a common association (3,6).…”

“…The main symptoms of IPF, including progressive dyspnea and dry cough, are often nonspecific (6). Frequent signs on physical examination include the presence of bilateral inspiratory crackles (Velcro-like) predominantly in the lower lung zones, and digital clubbing (3,5). Pulmonary function tests (PFTs) in IPF are characterized by a restrictive pattern combined with a decreased diffusing capacity.…”

“…Surgical lung biopsy (SLB) is the diagnostic method of choice for patients whose imaging findings are not definitive for UIP, including findings of probable UIP, indeterminate for UIP and alternative diagnosis. Hence, an integrated multidisciplinary approach assessing both imaging and pathological features is essential to establish a final diagnosis (3,6).…”

Consolidating in the present, with an eye to the future

“…There has also been a significant increase in the number of JBP articles related to invasive procedures, 7 , 8 as well as to the field of thoracic surgery, 9 clearly indicating an expansion of the representativeness of the Journal. In parallel, JBP review articles have sought to illuminate subjects of more general interest to our readership, such as sleep, 10 idiopathic pulmonary fibrosis, 11 and imaging, 12 together with special sections on continuing education in scientific methodology and imaging, with the goal of making the JBP an accessible tool for keeping everyone with an interest in respiratory medicine up to date.…”