Granulomatosis with polyangiitis (Wegener granulomatosis) is rare and high-mortality disease which occurs approximately for 3-16 per milion persons per year and affects mostly young males. It may involve many different systems, but involvement of the central nervous system occurs only in 7-11% of patients and is considered as threatening condition.
We report a rare case of a 55 years-old man diagnosed with granulomatosis with polyangiitis 8 years before involvement of central nervous system. Initial diagnosis was established by using histophathological findings of sinonasal masses and positive anti-neutrophil cytoplasmic antibodies with titer 1:40. Clinical remission was reached with prednisolone and azathioprine. 4 years later patient complained of general weakness, strong headache, especially, in forehead, subfebrile temperature and hearing disturbance. Ethmoidotomy was performed and effective. 4 years after that head magnetic resonance imaging showed invasive granuloma from ethmoidal cells into frontal lobes of brain. Computer tomography of chest showed small granuloma on right lung.
C reactive protein was 11.2 mg/L. Patient was treated with methylprednisolone (12 mg/day) and infusions of cyclophosphamide (1000 mg/day). 1 year later expansion in brain was stopped but treatment with azathioprine (100 mg/day) and methylprednisolone (16 mg/day) continues.
Spreading of vasculitis from paranasal sinuses into the central nerve system caused a severe and dangerous condition.
Our patient represents a rare and treatment-resistant involvement of the central nerve system in granulomatosis with polyangiitis, however, it can be stopped with intensive immunosuppressive treatment.
