Case report: Cerebral amyloid angiopathy-related inflammation in a patient with granulomatosis with polyangiitis.

Autor:	Seifert RM; Universitätsklinik für Neurologie, Evangelisches Klinikum Bethel, Bielefeld, Germany., Rauch M; Universitätsklinik für Neurologie, Evangelisches Klinikum Bethel, Bielefeld, Germany., Klingebiel R; Institut für diagnostische und interventionelle Neuroradiologie, Evangelisches Klinikum Bethel, Bielefeld, Germany., Boese LM; Institut für diagnostische und interventionelle Neuroradiologie, Evangelisches Klinikum Bethel, Bielefeld, Germany., Greeve I; Universitätsklinik für Neurologie, Evangelisches Klinikum Bethel, Bielefeld, Germany., Rudwaleit M; Universitätsklinik für Innere Medizin und Rheumatologie, Klinikum Bielefeld Rosenhöhe, Bielefeld, Germany., Schäbitz WR; Universitätsklinik für Neurologie, Evangelisches Klinikum Bethel, Bielefeld, Germany.
Jazyk:	angličtina
Zdroj:	Frontiers in neurology [Front Neurol] 2023 Nov 09; Vol. 14, pp. 1277843. Date of Electronic Publication: 2023 Nov 09 (Print Publication: 2023).
DOI:	10.3389/fneur.2023.1277843
Abstrakt:	Background: Cerebral amyloid angiopathy-related inflammation (CAA-ri) defines a subacute autoimmune encephalopathy, which is presumably caused by increased CSF concentrations of anti-Aβ autoantibodies. This autoinflammatory reaction is temporally and regionally associated with microglial activation, inflammation and radiological presence of vasogenic edema. Clinical characteristics include progressive demential development as well as headache and epileptic seizures. In the absence of histopathologic confirmation, the criteria defined by Auriel et al. allow diagnosis of probable resp. possible CAA-ri. CAA-ri shows responsiveness to immunosuppressive therapies and a possible coexistence with other autoinflammatory diseases. Methods: We present a case report and literature review on the diagnosis of CAA-ri in a patient with known granulomatosis with polyangiitis (GPA). Results: Initially, the presented patient showed neuropsychiatric abnormalities and latent arm paresis. Due to slight increase in CSF cell count, an initial antiviral therapy was started. MR tomography showed a pronounced frontotemporal edema as well as cerebral microhemorrhages, leading to the diagnosis of CAA-ri. Subsequent high-dose steroid treatment followed by six intravenous cyclophosphamide pulses resulted in decreased CSF cell count and regression of cerebral MRI findings. Conclusion: The symptoms observed in the patient are consistent with previous case reports on CAA-ri. Due to previously known GPA, we considered a cerebral manifestation of this disease as a differential diagnosis. However, absence of pachymeningitis as well as granulomatous infiltrations on imaging made cerebral GPA less likely. An increased risk for Aβ-associated pathologies in systemic rheumatic diseases is discussed variously. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2023 Seifert, Rauch, Klingebiel, Boese, Greeve, Rudwaleit and Schäbitz.)
Databáze:	MEDLINE
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