Granulomatosis with polyangiitis (CNS manifestations)
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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.
Epidemiology
CNS manifestations of GPA occur only in ~5% (range 2-9%) of the patients 1,2.
Pathology
GPA manifests in the CNS as 1,2:
- cerebral or meningeal granulomatous lesions (hypertrophic pachymeningitis 3
-4,4) - small vessel CNS vasculitis causing infarcts and arterial occlusion
- intracranial haemorrhage
- continuous invasion of an extracranial granuloma
-<li>cerebral or meningeal granulomatous lesions (<a title="Hypertrophic pachymeningitis" href="/articles/hypertrophic-pachymeningitis">hypertrophic pachymeningitis</a> <sup>3-4</sup>)</li>- +<li>cerebral or meningeal granulomatous lesions (<a href="/articles/hypertrophic-pachymeningitis">hypertrophic pachymeningitis</a> <sup>3,4</sup>)</li>
References changed:
- 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. <a href="https://doi.org/10.1007/s00415-017-8416-0">doi:10.1007/s00415-017-8416-0</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28220286">Pubmed</a> <span class="ref_v4"></span>
- 4. Smoleńska Ż, Masiak A, Zdrojewski Z. Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis. (2018) Reumatologia. 56 (6): 399-405. <a href="https://doi.org/10.5114/reum.2018.80719">doi:10.5114/reum.2018.80719</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30647488">Pubmed</a> <span class="ref_v4"></span>
- 3. https://www.ncbi.nlm.nih.gov/pubmed/28220286
- 4. https://www.ncbi.nlm.nih.gov/pubmed/30647488
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