Wegener's granulomatosis

In this condition, glomerulonephritis occurs together with necrotizing granulomatous lesions affecting the nasopharynx, lungs and kidneys. The necrotizing glomerular lesions do not appear to be due to immune complex deposition. PR3-ANCA positivity is the rule.

Treatment of Wegeners granulomatosis

The sooner treatment is instituted the more chance there is of recovery of renal function. Corticosteroids and cyclophosphamide are of benefit: pulsed high-dose methylprednisolone and plasmapheresis may reverse advanced disease.

Once remission has been achieved, azathioprine or mycophenolate mofetil may be substituted for cyclophosphamide.

Relapse after complete cessation of immunosuppressive therapy has been observed relatively frequently, and there is now a consensus that long-term, albeit relatively low-dose, immunosuppression is appropriate.

Long-term follow-up of patients is mandatory. Intravenous immunoglobulin therapy shows promise in the treatment of severe and drug-resistant cases.

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