Granulomatosis with Polyangitis (Wegener’s) and Central Nervous System Involvement: Case Report

Abstract

Introduction Granulomatosis with polyangitis (Wegener’s) is an antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis, which commonly involves the upper and lower respiratory tracts and kidneys. Central nervous system involvement is reported in less than 11%, and rarely present at onset.

Case Outline We report the case of a 41-year-old male patient with a high disease activity, large organ involvement, as well as central nervous system manifestations presented at onset. Treatment with intravenous pulse methylprednisolone, followed by the pulsed doses of cyclophosphamide was induced. After 6 months of cyclophosphamide pulse therapy a remission was achieved. Next, azathioprine was used for maintenance during the next 18 months. There were no disease flares during 24-month follow-up.

Conclusion Granulomatosis with polyangitis (Wegener’s) with large organ involvement, affecting the central nervous system structures require a rapid diagnosis and intensive medication treatment in order to prevent or reduce irreversible damage. Our experience confirms the findings reported in the literature that the severe forms of the disease are associated with increased probability of achieving remission, which reflects increased responsiveness of such patients to immunosuppressant therapy.

Keywords: granulomatosis polyangitis (Wegener’s); central nervous system; treatment; outcome