Öz
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.
Anahtar Kelimeler
Idiopathic pulmonary fibrosis usual interstitial pneumonia antifibrotic drugs
Kaynakça
- 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.
Öz
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.
Anahtar Kelimeler
idiyopatik pulmoner fibrozis olağan interstisyel pnömoni antifibrotik tedavi
Kaynakça
- 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.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Göğüs Hastalıkları |
| Bölüm | Review [en] Derleme [tr] |
| Yazarlar | |
| Yayımlanma Tarihi | 29 Şubat 2024 |
| Gönderilme Tarihi | 11 Ocak 2024 |
| Kabul Tarihi | 25 Ocak 2024 |
| Yayımlandığı Sayı | Yıl 2024 Cilt: 5 Sayı: 1 |
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