Di Muzio B, Sharma R, Geravandi M, et al. Granulomatosis with polyangiitis (CNS manifestations). Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-40104
Granulomatosis with polyangiitis - CNS manifestations
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.
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.
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
3. Choi HA, Lee MJ, Chung CS. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis. (2017) Journal of neurology. 264 (4): 724-732. doi:10.1007/s00415-017-8416-0 - Pubmed
4. Smoleńska Ż, Masiak A, Zdrojewski Z. Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis. (2018) Reumatologia. 56 (6): 399-405. doi:10.5114/reum.2018.80719 - Pubmed
5. Guzman-Soto M, Kimura Y, Romero-Sanchez G et al. From Head to Toe: Granulomatosis with Polyangiitis. Radiographics. 2021;41(7):1973-91. doi:10.1148/rg.2021210132 - Pubmed