Cryptogenic fibrosing alveolitis: have we made any progress?

The interstitial lung diseases (ILD) are a heterogeneous group of disorders the most common of which is cryptogenic fibrosing alveolitis (CFA). This article has summarized recent work in particular on the diagnosis, pathogenesis and treatment of CFA, by reviewing published data accessed through Medline searching. Recent reports suggest a higher prevalence of CFA than previously documented (13.2-20.2/100,000 population) and a rising mortality rate. The prognosis is universally poor with 50% of patients dying within 5 years. Although approximately 30% of patients may live for long periods (> 10 years), morbidity is significant and quality of life in the long-term survivors is poor. Diagnosis is traditionally based on an open lung biopsy, however, more recently the high resolution computer tomography (HRCT) is often used; however, its use without a tissue diagnosis remains controversial. In conclusion, we know substantially more about the pathogenesis of the disease and from this work have a number of possibilities for new therapeutic strategies that will hopefully reach the bedside in the near future. Additionally we have some new non-invasive tests that offer hope for stratifying patients but require further evaluation. For assessing both therapy and investigations we will need substantial groups of patients in multicentre studies to provide sufficient power to allow a conclusion to be reached. To ensure any further progress we must collaborate and enter our patients into such trials.