Granulomatosis with polyangiitis (CNS manifestations)
Citation, DOI, disclosures and article data
At the time the article was created Bruno Di Muzio had no recorded disclosures.View Bruno Di Muzio's current disclosures
At the time the article was last revised Rohit Sharma had no recorded disclosures.View Rohit Sharma's current disclosures
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: upper respiratory tract manifestations
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.
On this page:
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:
cerebral or meningeal granulomatous lesions (hypertrophic pachymeningitis)
small vessel CNS vasculitis causing infarcts and arterial occlusion
continuous invasion of an extracranial granuloma
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