CNS manifestations of granulomatosis with polyangiitis (GPA) are rare.
For a general discussion of the condition, please refer to the main article on granulomatosis with polyangiitis (GPA). For other organ-specific radiographic features, please refer to individual articles:
Granulomatosis with polyangiitis (previously known as Wegener granulomatosis), is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting medium to small-sized arteries, capillaries, and veins.
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Epidemiology
CNS manifestations of granulomatosis with polyangiitis occur only in ~5% (range 2-9%) of the patients 1,2.
Pathology
Granulomatosis with polyangiitis manifests in the CNS as 1-5:
cerebral or meningeal granulomatous lesions (hypertrophic pachymeningitis)
small vessel CNS vasculitis causing infarcts and arterial occlusion
continuous invasion of an extracranial granuloma
Radiographic features
There are no pathognomonic radiographic features of CNS granulomatosis with polyangiitis. Manifestations such as hypertrophic pachymeningitis, CNS vasculitis, and intracranial hemorrhage have imaging characteristics that are shared by other etiologies of those entities 5, thus, see those individual articles for detailed discussion.
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