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Last year in November, a 58-year-old man was referred to our hospital for further diagnosis and treatment. He presented with prolonged fever which further developed into severe haemoptysis and he subsequently died. This patient had a very interesting clinical presentation over the period of his sickness. We believe that reviewing this case will help all clinicians to be more aware of the importance of early recognition and understanding the time variable development of this disease; earlier treatment may result in a better outcome.
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2. Kallenberg CGM. Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun 2008;30:29-36.
3. Kallenberg CGM, Heeringa P, Stegeman CA. Mechanisms of disease: pathogenesis and treatment of ANCA-associated vasculitis. Nat Clin Pract Rheumatol 2006;2:661-70.
4. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009;68:310-7.
5. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol 2008;26:94-104.
6. Fauci AS, Haynes BS, Katz P, et al. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76-85.
7. Frankel SK, Cosgrove GP, Fischer A. Update in the diagnosis and management of pulmonary vasculitis. Chest 2006;129:452-65.
8. DeGroot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 2009;150 :670-80.
9. DeGroot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibodyassociated vasculitis. Arthritis Rheum 2005;52:2461-9.
10. Walsh M, Merkel PA, Mahr A, et al. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis. Arthritis Care Res (Hoboken) 2010;62 :1166-73.
11. Sanders JS, Slot MC, Stegeman CA. Maintenance theroapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349: 2072-3.
12. Bosch X, Guilabert A, Espinosa G, et al. Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. JAMA 2007;298:655-69.
13. Martinez V, Cohen P, Pagnoux C, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum 2008;58 :308-71.
14. Joy MS, Hogan SL, Jennette JC, et al. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 2005;20:2725-32.
15. Wegener’s Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener’s Granulomatosis. N Engl J Med 2005;352 :351-61.
16. Walsh M, Chaudhry A, Jayne D. Long-term follow-up of relapsing/refractory anti-neutrophil cytoplasm antibody associated vasculitis treated with the lymphocyte depleting antibody alemtuzumab (CAMPATH-1H). Ann Rheum Dis 2008;67:1322-7.
17. Mukhtyar C, Flossmann O, Hellmich B, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis 2008;67:1004-10.
18. Hellmich B, Lamprecht P, Gross WL. Advances in the therapy of Wegener’s granulomatosis. Curr Opin Rheumatol 2006;18:25-32.
19. Seo P. Wegener’s granulomatosis: managing more than inflammation. Curr Opin Rheum 2008;20:10-6.