There is no evidence that steroids alter the long-term course of the disease. A have been used with apparent benefit. Dapsone has been used with variable results in several series. The course is unpredictable, with about 55 per cent of subjects surviving for 10 years. Infection and systemic vasculitis caused more deaths than did airway obstruction in a recent series.
Remissions do occur.
Aortic valve disease has required surgery.