The diagnosis and treatment of idiopathic pulmonary fibrosis

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.

Keywords

• Idiopathic pulmonary fibrosis
• usual interstitial pneumonia
• antifibrotic drugs

İdiyopatik pulmoner fibrozisin tanı ve tedavisi

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.

Keywords

• idiyopatik pulmoner fibrozis
• olağan interstisyel pnömoni
• antifibrotik tedavi

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