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J. H. Stone, P. A. Merkel, R. Spiera, P. Seo, C. A. Langford, G. S. Hoffman, U. Specks, et al., “RAVE-ITN Research Group,” The New England Journal of Medicine, Vol. 5, No. 363, 2010, pp. 221-232. doi:10.1056/NEJMoa0909905

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• TITLE: A Fatal Case of Treatment-Related Adverse Effects in Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)

  AUTHORS: Marina Magrey, Maria Antonelli

  KEYWORDS: Granulomatosis with Polyangiitis, Wegener’s Granulomatosis, Cerebrovascular Accident, Infection, CNS Manifestation

  JOURNAL NAME: Open Journal of Rheumatology and Autoimmune Diseases, Vol.1 No.2, November 29, 2011

  ABSTRACT: Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.