ABSTRACT
Oral corticosteroids are tried in virtually all cases. However, they are successful in only about 40% of patients. Initially, 60 mg prednisone is given daily for four weeks and then, gradually, tailed down to 20 mg daily if there is a response. Alternatively, cyclophosphamide may be given daily with prednisone on alternate days. Steroids are most likely to work in the following instances: • young patients who are not breathless; • active alveolitis on bronchoalveolar lavage, gallium scan or EDTA
clearance; • desquamative interstitial pneumonia (DIP) histology 60% response; • usual interstitial pneumonia (UIP) histology only 12% response; • little fibrosis present. To assess the response to therapy, the patient is seen regularly in the clinic with repeat lung function tests and chest X-ray. EDTA clearance (or gallium scanning) may be repeated, as may fine cut CT scanning. Options for treatment include: • lung transplantation for younger, fitter patients; • oxygen for the chronically hypoxic.
