Granulomatosis with polyangiitis (CNS manifestations)

Last revised by Rohit Sharma on 13 Dec 2022

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.

CNS manifestations of granulomatosis with polyangiitis occur only in ~5% (range 2-9%) of the patients 1,2.

Granulomatosis with polyangiitis manifests in the CNS as 1-5:

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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