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Treatment of idiopathic pulmonary fibrosis: Is there anything new?
Author(s) -
ABDELAZIZ Muntasir M.,
SAMMAN Yaseen S.,
WALI Siraj O.,
HAMAD Mahir M. A.
Publication year - 2005
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/j.1440-1843.2005.00712.x
Subject(s) - medicine , pirfenidone , idiopathic pulmonary fibrosis , pulmonary fibrosis , azathioprine , interstitial lung disease , pulmonary function testing , cyclophosphamide , usual interstitial pneumonia , disease , etiology , lung , chemotherapy
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrosing interstitial pneumonia of unknown aetiology and is associated with the histological picture of usual interstitial pneumonia. Treatment in most cases is unsatisfactory and the prognosis remains poor. There is insufficient evidence to suggest that any treatment, apart from lung transplantation, improves survival or halts disease progression for IPF patients. Data on treatment response are limited by the paucity of clinical trails, the lack of homogenous clinical features, the small number of patients, and the absence of histological and radiological documentation in many cases. Anti‐inflammatory medications such as corticosteroids, azathioprine and cyclophosphamide remain the commonly used medications. More recently, it has been proposed that IPF is a primary fibrotic disease rather than an inflammatory condition. Antifibrotic agents such as colchicine, pirfenidone and interferon‐gamma (IFN‐γ) have been tried. However, a recent placebo‐controlled trial has failed to demonstrate a significant effect of IFN‐γ on disease progression, lung function or quality of life in IPF patients, though a clinically significant survival benefit of the drug could not be ruled out.