Idiopathic pulmonary fibrosis: new evidence and an improved standard of care in 2012

Abstract

1Although these recommendations were based on the consensus of an expert panel and limitations of the data were acknowledged, a combination of prednisone and azathioprine or cyclo phosphamide was suggested as an appropriate treatment regimen for idiopathic pulmonary fi brosis. Worldwide, treatment with combined prednisone and azathioprine became standard care—essentially on the basis of fi ndings from an early study. 2 The IFIGENIA trial 3 showed that the addition of N-acetyl cysteine to a regimen of prednisone plus azathioprine (pred-aza-NAC) preserved forced vital capacity and the diff using capacity of the lung for carbon monoxide compared with prenisone plus azathioprine in 1 year; therefore, the standard of care for treatment of idiopathic pulmonary fi brosis changed to pred-aza-NAC from 2005. Sub stantial evidence accumulated after publication of the 2000 recommendations 1 led to robust grading of the evidence and the development of new evidencebased guidelines. 4,5