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[An autopsy case of P-ANCA-positive microscopic polyangiitis with multiple cerebral hemorrhagic infarction]
A Sasaki1, J Hirato, Y Nakazato
1First Department of Pathology, Gunma University School of Medicine, Japan.
Abstract:
A 78-year-old man who had presented with a left hemiparesis was hospitalized and diagnosed as having cerebral infarction. He developed a rapidly progressive glomerulonephritis and a massive hemorrhage of the alimentary tract, and was serologically positive for perinuclear pattern antineutrophil cytoplasmic autoantibody (p-ANCA). He passed away 19 days after the onset of the illness. The autopsy diagnosis was microscopic polyangiitis based on the histological findings of necrotizing angiitis of the small vessels, necrotizing and crescentic glomerulonephrits and alveolar hemorrhage of the lung. Neuropathological examination revealed multiple hemorrhagic infarction of the cerebral cortex caused by necrotizing angiitis. In the literature, central neurological disorders are rare in p-ANCA related vasculitis. In the case of p-ANCA related vasculitis, however, there is the possibility of cerebral infarction seen as in this case.
Insights
Microscopic polyangiitis, a rare vasculitis, can cause severe neurological issues like cerebral infarction. This case highlights the potential for central nervous system involvement in perinuclear pattern antineutrophil cytoplasmic autoantibody-associated vasculitis.
Area of Science:
- Nephrology
- Neurology
- Rheumatology
Background:
- Microscopic polyangiitis (MPA) is a small-vessel vasculitis often associated with perinuclear pattern antineutrophil cytoplasmic autoantibodies (p-ANCA).
- While MPA commonly affects the kidneys and lungs, central nervous system (CNS) involvement is considered rare.
Observation:
- A 78-year-old male presented with left hemiparesis, diagnosed with cerebral infarction.
- He subsequently developed rapidly progressive glomerulonephritis, massive gastrointestinal hemorrhage, and tested positive for p-ANCA.
- Autopsy confirmed MPA, revealing necrotizing angiitis, crescentic glomerulonephritis, and alveolar hemorrhage.
Findings:
- Neuropathological examination identified multiple hemorrhagic cerebral cortical infarcts attributed to necrotizing angiitis.
- This case demonstrates a rare manifestation of CNS vasculitis in the context of p-ANCA positivity.
Implications:
- The findings suggest that cerebral infarction should be considered in the differential diagnosis of neurological complications in patients with p-ANCA-associated vasculitis.
- This case underscores the importance of recognizing the potential for severe neurological sequelae in MPA.
- Further research into the mechanisms and prevalence of CNS involvement in ANCA-associated vasculitis is warranted.