Current Status and New Treatment Approaches in Idiopathic Pulmonary Fibrosis

Yıl 2024, , 91 - 103, 30.08.2024

Seyde Nur Uçar , Yusuf Elma , Bülent Altınsoy , Ayşegül Tomruk Erdem , Emine Yılmaz Can

https://doi.org/10.29058/mjwbs.1514437

Öz

Idiopathic pulmonary fibrosis is a progressive lung disease of unknown etiology, characterized by the replacement of normal lung tissue with connective tissue that does not allow for gas exchange. The development of fibrosis in lung tissue is a chronic process and multiple signaling pathways and mediators contribute to this process. Despite its unknown etiology, several genetic and environmental factors have been associated with idiopathic pulmonary fibrosis. The clinical presentation of idiopathic pulmonary fibrosis involves a gradual loss of lung function and patients usually die in the end-stage with respiratory failure. Therefore, it is critical to identify and implement appropriate treatment approaches.While there is no definitive cure, several treatment options are available to slow disease progression and improve quality of life. Among the current treatment approaches, the most commonly used are antifibrotic drugs such as pirfenidone and nintedanib. These medications can slow the progression of fibrosis in lung tissue and relieve symptoms. New therapeutic approaches targeting signaling pathways and mediators involved in pathophysiology are being developed for the disease, for which there is no effective treatment yet. These approaches include potential therapies such as the combination of pirfenidone and nintedanib, BI 1015550, PLN-74809, TRK-250, BMS-986278, PBI-4050, TD139, treprostinil and stem cell therapy. Through current and future studies, it is hoped that more effective treatment methods can be developed and the disease can be completely cured.

Anahtar Kelimeler

Antifibrotic drugs, clinical trials, idiopathic pulmonary fibrosis, new treatment approaches, pulmonary fibrosis

İdiopatik Pulmoner Fibroziste Mevcut Durum ve Yeni Tedavi Yaklaşımları

Öz

İdiyopatik pulmoner fibrozis normal akciğer dokusunun yerini gaz değişimine olanak vermeyen bağ dokusunun aldığı, etyolojisi bilinmeyen, ilerleyici bir akciğer hastalığıdır. Akciğer dokusunda fibrozisin gelişimi kronik bir süreçtir ve bu sürece birden fazla sinyal yolağı ve mediyatör katkıda bulunmaktadır. Bilinmeyen etiyolojisine rağmen, çeşitli genetik ve çevresel faktörler idiopatik pulmoner fibrozisle ilişkilendirilmektedir. İdiopatik pulmoner fibrozisin kliniği, akciğer fonksiyonlarının kademeli kaybını içermekte ve hastalar son evrede genellikle solunum yetmezliği ile kaybedilmektedir. Bu nedenle, uygun tedavi yaklaşımlarının belirlenmesi ve uygulanması kritik bir öneme sahiptir. Kesin bir tedavisi olmamakla birlikte, hastalığın ilerlemesini yavaşlatmak ve yaşam kalitesini artırmak için çeşitli tedavi seçenekleri bulunmaktadır. Mevcut tedavi yaklaşımları arasında en yaygın kullanılanlar, pirfenidon ve nintedanib gibi antifibrotik ilaçlardır. Bu ilaçlarla, akciğer dokusunda fibrozisin ilerleyişi yavaşlatılabilmekte ve semptomlar hafifletilebilmektedir. Henüz etkin bir tedavisi olmayan hastalık için, patofizyolojide rol oynayan sinyal yolaklarının ve mediyatörlerin hedeflendiği yeni tedavi yaklaşımları oluşturulmaktadır. Bu yaklaşımlar arasında pirfenidon ve nintedanib kombinasyonu, BI 1015550, PLN-74809, TRK-250, BMS-986278, PBI-4050, TD139, treprostinil ve kök hücre tedavisi gibi potansiyel tedaviler yer almaktadır. Şu anda yürütülen ve gelecekte gerçekleştirilecek çalışmalar sayesinde, daha etkili tedavi yöntemlerinin geliştirilmesi ve hastalığın tam anlamıyla tedavi edilebilmesi umut edilmektedir.

Anahtar Kelimeler

Antifibrotik ilaçlar, idiopatik pulmoner fibrozis, klinik çalışmalar, pulmoner fibrozis, yeni tedavi yaklaşımları

Kaynakça

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