Staging of idiopathic pulmonary fibrosis

Author: 
Tomoo Kishaba

Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology (1). Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition (2). Majority of IPF patients have been elderly men with smokers (3).The older, the more often have IPF (4).

Clinical point of view, non-productive cough, and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing key findings (5).Serum marker such as lactate dehydrogenase (LDH), Krebs van den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test (PFT) and 6-minute walk test (6MWT) are quite meaningful physiological examination. International IPF guideline published recently and highlighted on the importance of high-resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. However, baseline clinical physiological status can predict future prognosis of IPF. Recently some composite index were proposed for mortality prediction of IPF patients.In management, two anti-fibrotic agents such as pirfenidone and nintedanib are available for prevention of progression of IPF. In this review, I focus on clinical characteristics, staging and real management of IPF including comorbidities.

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DOI: http://dx.doi.org/10.24327/ijcar.2017.3011.0164