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. 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.