Review of cryptogenic fibrosing alveolitis, including current treatment guidelines.

Cryptogenic fibrosing alveolitis (CFA), known as idiopathic pulmonary fibrosis in the USA, is characterised by inflammation and fibrosis of the alveoli and interstitium of the lungs, favouring the subpleural and basal regions. By definition the diagnosis demands that all known causes of pulmonary fibrosis have been excluded. It is now clear that the CFA population is comprised of a heterogeneous group, with varying clinical, radiological and histological features, which in turn may be used to predict response to treatment and prognosis. The British Thoracic Society has recently produced guidelines on the investigation and management of diVuse parenchymal lung disease, including CFA.