The diagnosis of idiopathic pulmonary fibrosis can be made only after
exclusion of other entities such as neoplasm, toxic treatments, collagen
vascular diseases, occupational exposure or granulomatous diseases, such as
sarcoidosis. The repercussions on gas exchanges are the most reliable
indications of the severity of the disease, the measure of lung volume or
chest x-rays alone often being misleading. Biopsies obtained during
transbronchial procedures by thoracoscopies or thoracotomy are of great
help, but mainly to rule out other diseases. In many cases, only a high
resolution computerised tomography (CT) scan and bronchoalveolar lavage are
performed to rule out infection or tumour and to assess the inflammatory
state of the disease. Due to the fact that barely a quarter of patients
respond to corticosteroids alone, cytostatics (in particular azathioprine)
are often prescribed simultaneously with low dose corticosteroids, either
initially or after an unsuccessful trial of corticosteroids. Cyclosporin has
been useful for only a limited number of patients. Colchicine has been shown
useful in an open trial but its role still needs to be assessed.
Anticytokine therapy and the role of substances such as relaxin are still at
the experimental stage. Lung transplantation is now a therapeutic option for
selected patients.