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The diagnosis and treatment of idiopathic pulmonary fibrosis

Year 2024, , 85 - 90, 29.02.2024
https://doi.org/10.47582/jompac.1418227

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease (ILD) of unknown cause. The prognosis of IPF is poor, respiratory failure is the most common cause of mortality. Velcro rales are typical on respiratory system examination. Clubbing is seen in 30-60% of IPF cases. There is no laboratory test specific to IPF. Usual interstitial pneumonia (UIP) pattern is seen in IPF. UIP features in high-resolution computed tomography (HRCT); peripheral subpleural bibasilar reticular opacities, honeycombing, traction bronchiectasis and interseptal thickening. It shows craniocaudal localization. Diagnosis of IPF; It is diagnosed by the combination of HRCT findings and clinical findings. Antifibrotic drugs (Pirfenidone and Nintedanib) slow down the progression of IPF and reduce the number of annual attacks and reduce the frequency of hospitalization.

References

  • Raghu G, Collard HR, Egan JJ, et al. An official A TS/ERS/JRS/ALA T statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
  • Esposito DB, Lanes S, Donneyong M, et al. Idiopathic pulmonary fibrosis in United States automated claims. incidence, prevalence, and algorithm validation. Am J Respir Crit Care Med. 2015;192(10):1200-1207.
  • Maher TM, Bendstrup E, Dron L, et al.Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021;22(1):197.
  • Maher TM. Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. Clin Chest Med. 2012;33(1):69-83.
  • Sellarez J, Hernandez-Gonzales V, Lucena C, et al. Auscultation of velkro cracles is assiciated with usual intertitial pneumonia. Medicine. 2016;95(5):e2573.
  • Molyneaux PL, Maher TM. The role of infection in the pathogenesis of idiopathic pulmonary fibrosis. Eur Respir Rev. 2013;22(129):376-381.
  • King TE. Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis. UpToDate. 2023. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-idiopathic-pulmonary-fibrosis Jan 26, 2024.
  • Musellim B, Okumus G, Uzaslan E, et al. Epidemiology and distribution of interstitial lung diseases in Turkiye. Clin Respir J. 2014;8(1):55-62.
  • Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44.
  • Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47.
  • Hutchinson J, Fogarty A, Hubbard R, McKeever T. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015;46(3):795-780.

İdiyopatik pulmoner fibrozisin tanı ve tedavisi

Year 2024, , 85 - 90, 29.02.2024
https://doi.org/10.47582/jompac.1418227

Abstract

İdiyopatik pulmoner fibrozis (İPF), bilinmeyen nedenlere dayanan kronik, ilerleyici fibrozis gösteren bir interstisyel akciğer hastalığıdır (İAH). İPF'nin prognozu kötüdür, solunum yetmezliği en yaygın ölüm nedenidir. Velcro ralleri, solunum sistemi muayenesinde tipiktir. İPF vakalarının %30-60'ında çomak parmak görülür. İPF'ye özgü bir laboratuvar testi bulunmamaktadır. İPF'de tipik olarak usual interstisyel pnömoni (UIP) deseni görülür. Yüksek çözünürlüklü bilgisayarlı tomografi (YÇBT) ile UIP özellikleri; periferik subplevral bibazal retiküler opasiteler, petekleşme, çekme bronşiektazisi ve interseptal kalınlaşma içerir. Bu, kraniokaudal lokalizasyon gösterir. İPF'nin tanısı; YÇBT bulguları ile klinik bulguların birleşimiyle konur. Antifibrotik ilaçlar (Pirfenidon ve Nintedanib), İPF'nin ilerlemesini yavaşlatır, yıllık atak sayısını azaltır ve hastaneye yatış sıklığını düşürür.

References

  • Raghu G, Collard HR, Egan JJ, et al. An official A TS/ERS/JRS/ALA T statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
  • Esposito DB, Lanes S, Donneyong M, et al. Idiopathic pulmonary fibrosis in United States automated claims. incidence, prevalence, and algorithm validation. Am J Respir Crit Care Med. 2015;192(10):1200-1207.
  • Maher TM, Bendstrup E, Dron L, et al.Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021;22(1):197.
  • Maher TM. Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. Clin Chest Med. 2012;33(1):69-83.
  • Sellarez J, Hernandez-Gonzales V, Lucena C, et al. Auscultation of velkro cracles is assiciated with usual intertitial pneumonia. Medicine. 2016;95(5):e2573.
  • Molyneaux PL, Maher TM. The role of infection in the pathogenesis of idiopathic pulmonary fibrosis. Eur Respir Rev. 2013;22(129):376-381.
  • King TE. Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis. UpToDate. 2023. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-idiopathic-pulmonary-fibrosis Jan 26, 2024.
  • Musellim B, Okumus G, Uzaslan E, et al. Epidemiology and distribution of interstitial lung diseases in Turkiye. Clin Respir J. 2014;8(1):55-62.
  • Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44.
  • Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47.
  • Hutchinson J, Fogarty A, Hubbard R, McKeever T. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015;46(3):795-780.
There are 11 citations in total.

Details

Primary Language English
Subjects Chest Diseases
Journal Section Review [en] Derleme [tr]
Authors

Berna Akıncı Özyürek 0000-0003-0206-7615

Publication Date February 29, 2024
Submission Date January 11, 2024
Acceptance Date January 25, 2024
Published in Issue Year 2024

Cite

AMA Akıncı Özyürek B. The diagnosis and treatment of idiopathic pulmonary fibrosis. J Med Palliat Care / JOMPAC / Jompac. February 2024;5(1):85-90. doi:10.47582/jompac.1418227

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