Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

Abstract

Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

Current Developments in Interstitial Lung Disease

“…Nationwide registries will provide much-needed data on IPF patients, which can assist researchers in overcoming the remaining barriers to understanding IPF pathophysiology and developing new medical therapies for this incurable disease. 18 …”

Exercise Improves Lung Inflammation, but Not Lung Remodeling and Mechanics in a Model of Bleomycin-Induced Lung Fibrosis

“…Idiopathic pulmonary fibrosis (IPF) is a devastating disease, leading to death normally between 3 and 5 years after the diagnostic [ 1 ]. The main symptoms include dyspnea and cough, reflecting the rapidly pulmonary remodeling, decrease of lung function, and subsequent permanent hypoxemia [ 2 ]. In addition, there is no curative treatment for IPF, and many studies emerged to ensure the survival and improve the quality of life of these patients, by preserving lung function and minimizing adverse effects of therapy [ 1 – 3 ].…”

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

“…The ATS/ERS/JRS/ALAT 2011 guidelines for IPF have assigned a primary diagnostic role to HRCT, and the HRCT criteria outlined was the same as those in the revised guideline published in 2015 [8][9][10]. The diagnosis of IPF should be based on the exclusion of other known causes of ILDs (environmental exposures, drugs, and CTDs) and presence of UIP on HRCT; UIP is characterized on HRCT by the presence of reticular abnormalities with subpleural and basal distribution, honeycombing with or without traction bronchiectasis, and absence of features inconsistent with UIP such as extensive ground-glass opacities, diffuse mosaic attenuation, profuse micronodules, and consolidations.…”

“…Bilateral inspiratory fine crackles, most prominent at the lung bases, are usually audible on auscultation. Clubbed fingers characterized by hypertrophy and enlargement of the distal phalanges of the hands are often seen and reported in 30-50% of patients with IPF (idiopathic pulmonary fibrosis; to be mentioned later) [8]. Clinical features suggestive of rheumatic disorders, such as arthralgia, Raynaud's phenomenon, and skin manifestations, might be observed in the setting of an underlying CTD.…”

“…High-resolution computed tomography (HRCT), which offers better definition of the characteristic details of lung parenchyma, can reveal usually bilateral, peripheral, and basilar predominant abnormalities with reticulonodular infiltrates, often with honeycombing and cystic changes by IP type to be described later. Although chest radiography is less useful than HRCT in the detailed evaluation, it is helpful for evaluating disease distribution and its serial change during patient follow up [8]. Physiologically, the patients demonstrate diminished diffusion capacity (decreased DLco) with usually restrictive impairment in pulmonary function tests.…”