CNS manifestations of granulomatosis with polyangiitis (GPA) (previously known as Wegener granulomatosis) are rare. Granulomatosis with polyangiitis is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins.
CNS manifestations of GPA occur only in ~5% (range 2-9%) of the patients 1,2.
GPA manifests in the CNS as 1,2:
- 1. De Luna G, Terrier B, Kaminsky P et-al. Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes. Rheumatology (Oxford). 2014;54 (3): 424-32. doi:10.1093/rheumatology/keu336 - Pubmed citation
- 2. Provenzale JM, Allen NB. Wegener granulomatosis: CT and MR findings. AJNR Am J Neuroradiol. 1996;17 (4): 785-92. Pubmed citation