Treatment

The following recommendations have emerged from consensus discussions and trials conducted by the European Vasculitis Study Group (EUVAS: www.vasculitis.org) [do not enable link]. Therapeutic regimens have been designed according to the severity and extent of disease at presentation (Table 3).

What to watch when using cyclophosphamide

		Daily oral administration has a higher incidence of side-effects compared with intermittent pulse dosing. But daily dosing has a reduced subsequent relapse rate. Daily oral cyclophosphamide may therefore be more effective for inducing remission in extensive disease.
		The recommended dose is reduced in the elderly. Some centres also reduce the dose in impaired renal function (Table 4).
		Cyclophosphamide should be continued until remission has been achieved. This is within 3 months in 70% of cases and six months in 90%. At this time substituting azathioprine for cyclophosphamide is as effective in preventing disease relapse as continuing cyclophosphamide.

Maintenance treatment should prevent relapse with minimal cumulative toxicity. A typical combination is azathioprine (1-2mg/kg of body weight/day) and prednisolone (5-10mg/day) continued for two to four years. The antibiotic sulfamethoxazole-trimethoprim (960mg twice a day) reduces relapse rates in Wegener's granulomatosis*.

Alternative remission maintenance drugs to azathioprine include:

		methotrexate;
		cyclosporin; and
		mycophenolate mofetil.

Alternative or additional induction treatments for remission, where standard regimens have failed include:

		intravenous immunoglobulin;
		plasma exchange;
		anti-thymocyte globulin; and
		mycophenolate mofetil.