

Etiology, Epidemiology, Patho physiology and Histo pathology of Idiopathic Pulmonary Fibrosis
Abstract
The progressive lung condition known as idiopathic pulmonary fibrosis (IPF) is characterised by lung scarring, especially from an unidentified source. The long-term prognosis for the disorder is dismal. IPF's classic symptoms include increasing dyspnoea, a dry, ineffective cough, and a slow development of shortness of breath. Restricted lung function impairment, decreased functional vital capacity, and decreased carbon monoxide diffusing capacity are typically linked to pulmonary function testing. Due to the disease's propensity to develop quickly in its later stages, early identification is crucial for optimal care. With a thorough evaluation of the patient's medical history, imaging findings, and elimination of other illnesses, the diagnosis may often be made without a biopsy. Whatever it is, when there is ambiguity about the diagnosis, a lung biopsy can be necessary.
In order to help diagnose IPF, imaging methods particularly computed tomography scans often show a characteristic pattern of fibrosis, which is usually shown along the edges of both lungs and more concentrated at the bases in a timely way. In situations when the diagnosis is unclear, a lung biopsy can help determine the diagnosis. Treatment options include supportive care, oxygen supplementation when necessary, antifibrotic drugs, and, in extreme situations, lung transplantation, which is the only cure. In addition to emphasising the importance of the interprofessional healthcare team in its evaluation, diagnosis, and management, this exercise provides a thorough explanation of the aetiology, epidemiology, clinical symptoms, diagnosis, and therapy of IPF in adults.
References
Martinez FJ, Collard HR, Pardo A, Raghu G, Richeldi L, Selman M, Swigris JJ, Taniguchi H, Wells AU. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017 Oct 20;3:17074. [PubMed]
Njock MS, Guiot J, Henket MA, Nivelles O, Thiry M, Dequiedt F, Corhay JL, Louis RE, Struman I. Sputum exosomes: promising biomarkers for idiopathic pulmonary fibrosis. Thorax. 2019 Mar;74(3):309-312. [PMC free article] [PubMed]
Walsh SLF, Calandriello L, Silva M, Sverzellati N. Deep learning for classifying fibrotic lung disease on high-resolution computed tomography: a case-cohort study. Lancet Respir Med. 2018 Nov;6(11):837-845. [PubMed]
Wells AU. Efficacy data in treatment extension studies of idiopathic pulmonary fibrosis: interpret with caution. Lancet Respir Med. 2019 Jan;7(1):7-8. [PubMed]
Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendía-Roldán I, Selman M, Travis WD, Walsh S, Wilson KC., American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018 Sep 01;198(5):e44-e68. [PubMed]
Mei Q, Liu Z, Zuo H, Yang Z, Qu J. Idiopathic Pulmonary Fibrosis: An Update on Pathogenesis. Front Pharmacol. 2021;12:797292. [PMC free article] [PubMed]
Günther A, Korfei M, Mahavadi P, von der Beck D, Ruppert C, Markart P. Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis. Eur Respir Rev. 2012 Jun 01;21(124):152-60. [PMC free article] [PubMed]
Heukels P, Moor CC, von der Thüsen JH, Wijsenbeek MS, Kool M. Inflammation and immunity in IPF pathogenesis and treatment. Respir Med. 2019 Feb;147:79-91. [PubMed]
Luppi F, Kalluri M, Faverio P, Kreuter M, Ferrara G. Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management. Respir Res. 2021 Apr 17;22(1):109. [PMC free article] [PubMed]
Bano A, Chaker L, Muka T, Mattace-Raso FUS, Bally L, Franco OH, Peeters RP, Razvi S. Thyroid Function and the Risk of Fibrosis of the Liver, Heart, and Lung in Humans: A Systematic Review and Meta-Analysis. Thyroid. 2020 Jun;30(6):806-820. [PubMed]
Refbacks
- There are currently no refbacks.