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- Cytokine biology
- innate immunity
- interstitial fibrosis
- macrophage biology
- opportunist lung infections
- pulmonary vasculitis
- sarcoidosis
Idiopathic pulmonary fibrosis (IPF) is a disease characterised by alveolar epithelial damage followed by an aberrant repair mechanism characterised by fibroblast foci and activated myofibroblasts.1 Despite an incidence of 7.4/100 000 person years which is increasing year on year and a median survival of only 2–3 years, there is paucity of evidence for effective therapy.2 The current British Thoracic Society guidelines weakly recommend N-acetylcysteine (NAC), prednisolone and azathioprine (based on the IFIGENIA—Idiopathic Pulmonary Fibrosis International Group Exploring N-Acetylcysteine I Annual—trial) whereas the more recent guidelines of the American Thoracic Society/European Respiratory Society recommend lung transplantation or participation in a clinical trial as treatment options.3–5
Increasing recognition of the clinical need for effective IPF therapy has finally led to a number of clinical trials evaluating potential anti-inflammatory and anti-fibrotic agents. IFIGENIA demonstrated that triple therapy with NAC, azathioprine and prednisolone was better than azathioprine and prednisolone in combination in preserving lung function in IPF patients, suggesting that NAC inclusion was strongly contributing to the benefits …
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Competing interests None.
Provenance and peer review Commissioned; internally peer reviewed.
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