Srpski arhiv za celokupno lekarstvo 2015 Volume 143, Issue 1-2, Pages: 83-86
https://doi.org/10.2298/SARH1502083T
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Granulomatosis with polyangitis (Wegener’s) and central nervous system involvement: Case report
Tomić-Lučić Aleksandra (Clinical Center of Kragujevac, Internal Clinic, Department of Rheumatology, Kragujevac)
Veselinović Mirjana (Clinical Center of Kragujevac, Internal Clinic, Department of Rheumatology, Kragujevac)
Pantović Suzana (Faculty of Medical Sciences, Kragujevac)
Petrović Dejan (Clinical Center of Kragujevac, Clinic for Nephrology and Urology, Kragujevac)
Živanović Sandra (Faculty of Medical Sciences, Kragujevac)
Milovanović Jasmina (Faculty of Medical Sciences, Kragujevac)
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