[Treatment of patients with Wegener's granulomatosis or ANCA-associated vasculitis].
Standard therapy of Wegener's granulomatosis is prednisolone in combination with cyclophosphamide. Cyclophosphamide is continued for 15-18 months after diagnosis. During therapy 26% of the patients have severe or life-threatening side effects, mostly infections. Furthermore, there are multiple long-term side effects such as an increased incidence of malignancies, especially bladder carcinoma. During the last 10 years the European vasculitis study group (www.vasculitis.org) organized several randomized clinical trials to determine optimal treatment. Recently, it was demonstrated in one of these trials that in patients with generalized ANCA-associated vasculitis (vasculitis associated with antineutrophil cytoplasmic autoantibodies) withdrawal of cyclophosphamide and substitution by azathioprine after remission did not increase the rate of relapse and was safe. Wegner's granulomatosis is not a fatal disease anymore. Sometimes, however, patients lose their job. Fortunately, this occurs only in a small percentage of patients.
Tervaert
J W
JW
Academisch Ziekenhuis, afd. Klinische en Experimentele Immunologie, Postbus 5800, 6202 AZ Maastricht. secretariaat-imm@immuno.unimaas.nl
Stegeman
C A
CA
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use; D012008:Recurrence; D012074:Remission Induction; D014657:Vasculitis / Q000188:drug therapy* / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine