Zehra YAŞAR, Erdoğan ÇETİNKAYA

İdiopatik pulmoner fibroziste güncel tedavi yaklaşımı

İdiopatik pulmoner fibroziste güncel tedavi yaklaşımıİdiopatik intersitisyel fibrozis (İPF), sebebi bilinmeyen ilerleyici, geri dönüşümsüz, histolojik ve radyolojik olarak olağan interstisyel pnömoni ile uyumlu, akciğerlerle sınırlı kronik interstisyel pnömonilerin bir formudur. Etyolojisi tam olarak aydınlatılamamıştır. Hastalığın gelişmesinde rol oynayan risk faktörleri sigara, ilerlemiş yaş, çevresel faktörler, viral infeksiyonlar, gastroösefajiyal reflü, ilaç maruziyeti ve genetik faktörler olarak sayılabilir. Hastalığın ortalama yaşı 65 olup, erkeklerde kadınlara göre daha sık görülür.Etyopatogenezinde daha önceleri ön planda kabul edilen inflamasyon teorisi son yıllarda yerini fibrozisle sonuçlanan yara iyileşmesi teorisine bırakmıştır. Tanı, interstisyel akciğer hastalığının bilinen diğer nedenlerinin dışlanması, yüksek çözünürlüklü bilgisayarlı tomografi (YÇBT)'de olağan interstisyel pnömoni (UIP) paterninin görülmesi ve biyopsi uygulanan hastalarda YÇBT ve biyopsi paternlerinin spesifik kombinasyonları ile konulur. Hastalığın şiddeti ve hasta tercihi tedavi yaklaşımının temelini oluşturur. Hastalar semptomlara, radyolojik ve solunum fonksiyon testlerine göre hafif, orta ve ağır olarak değerlendirilir. Hastalığın seyrinin öngörülmesi zordur uzun süren stabil dönemden sonra hastanın kliniğini kötüleştiren alevlenmeler görülebilir. Ortalama median sağkalım 3 yıldır. İleri yaşta tanı alma, sigara kullanımı, düşük vücut kitle indeksi, yaygın radyolojik tutulum, eşlik eden hastalıklar ve komplikasyonlar (pulmoner hipertansiyon, amfizem, bronkojenik kanser) sağkalımı azaltan nedenlerdir. Tedavide son yıllarda İPF patogenezinde öne sürülen mekanizmalara yönelik olarak özellikle de patolojik yolakta fibroblast birikimi ve myelofibroblastlara dönüşüm basamağını hedef alan ilaçlar olan pirfenidon ve nintedanib ile yapılan Faz 2 ve 3 çalışmalarında umut vadeden başarılı sonuçlar bildirilmiştir. Hafif ve orta şiddette İPF olarak değerlendirilen hastalar altta yatan karaciğer hastalığı yoksa ve bu ajanlara ulaşılabiliyorsa ilk tedavi olarak gecikmeden başlanması önerilmektedir. Nintedanib ile diare ve karaciğer fonksiyon testlerinde bozulma daha sık izlenirken pirfenidon da bulantı ve döküntü daha sık izlenir. Hasta güvenli klinik çalışmalara katılmak isterse yönlendirilmelidir ve geç kalınmadan transplantasyon programına alınmalıdır.

Current management of idiopathic pulmonary fibrosis

Current management of idiopathic pulmonary fibrosisIdiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and eventually fatal chronic interstitial pneumonia limited to the lung and associated with the histological and/or radiological Abant İzzet Baysal Üniversitesi Tıp Fakültesi, pattern of usual interstitial pneumonia.The mean age of the disease is 65 and it is more frequent in men than women. The disease was Göğüs Hastalıkları Anabilim Dalı, BOLU - TURKEYhistorically considered as an inflammatory disease, but currently this has shifted towards a prominent role of impaired wound healing process. The diagnosis of disease requires exclusion of other known causes of interstitial lung disease, the presence of a usual interstitial pneumonia pattern on high -resolution computed tomography (HRCT) in patients or specific combinations of HRCT and surgical lung biopsy patterns. The patients are considered as mild, moderate and severe according to the symptoms, radiological and pulmonary function tests. It is difficult to predict the course of the disease; clinical exacerbation can be seen after a long stable period. Average median survival is approximately 3 years. Advanced age, smoking, low body mass index, widespread radiological involvement, comorbidities and complications (pulmonary hypertension, emphysema, and bronchogenic cancer) are considered as poor prognostic factors. The treatment approach is the basis on the severity of the disease and patient preference. The recent positive result of the pirfenidone and nintedanib phase II and III clinical trials based on the prevailing mechanism of İPF pathogenesis particularly targeted fibroblast activation and myofibroblast differentiation have currently been reported. Initiating therapy with pirfenidone and nintedanib is recommended for the patients with mild and moderate İPF who do not have underlying liver disease and who live in area where these drugs are available. Nausea and rashes are more common in treatment with pirfenidone while diarrhea and deterioration in liver function tests are seen more frequently in nintedanib. The information regarding participation in randomized trials should be given to all patients and also early referral for transplantation should be considered.

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