Interstitial lung diseases are a heterogenic group of diseases who share in common manifestation of the disease process in the distal airways and alevolo-capilary membrane. Idiopathic pulmonary fibrosis (IPF) is of special interest as it is the most prevalent entity within the idiopathic interstitial pneumonias and it is associated with the worst prognosis. Diagnosis of IPF necessitates confirmation of a "usual interstitial pneumonia" (UIP) pattern on high-resolution computed tomography or on histology from a surgical lung biopsy. The recently published evidence-based IPF-guidelines for the first time define radiologic criteria for a UIP pattern and thus allow to make an IPF diagnosis in the absence of a surgical lung biopsy. Moreover, an IPF diagnosis can be made only after exclusion of any other cause for an interstitial lung disease.In the new IPF-guideline no medical therapy has received a positive recommendation. Based on the available evidence only pirfenidone has been shown to attenuate disease progression in mild to moderate IPF populations, with low-moderate quality of evidence. Pirfenidone has been approved for mild-moderate IPF in the European Union in February 2011 and is approved in Japan since 2009. In IPF-patients who cannot receive pirfenidone high-dose N-acetylcysteine is an "off-label-use" option that also has been shown to slow disease progression, however, based on low quality of evidence only. Recent studies suggest that anti-inflammatory therapies (prednione, azathioprine) may be harmful in IPF patients and cannot be recommended. Besides medical therapies IPF patients should receive best supportive care, which includes long-term oxygen therapy in hypoxemic patients and pulmonary rehabilitation as well as lung transplantation for eligible patients.
© Georg Thieme Verlag KG Stuttgart · New York.