Progressive cerebral infarction as the presenting feature of ANCA-associated vasculitis with concurrent Evans syndrome: a case report and literature review

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic autoimmune disease, and central nervous system involvement, though uncommon, can be a severe manifestation. The co-occurrence of AAV and Evans syndrome (ES) is exceptionally rare and may reflect broader immune dysregulation. A 72-year-old female presented with progressive right-sided hemiplegia and mixed aphasia. Imaging revealed multiple acute cerebral infarcts. Laboratory findings showed anemia, thrombocytopenia, acute kidney injury, and elevated inflammatory markers. Serology confirmed myeloperoxidase (MPO)-ANCA positivity, supporting a diagnosis of AAV, most consistent with microscopic polyangiitis. Subsequent hematological workup supported concurrent autoimmune cytopenias consistent with probable ES. Despite corticosteroids and intravenous immunoglobulin, her condition deteriorated with left middle cerebral artery occlusion; during hospitalization, this deterioration coincided with a SARS-CoV-2 infection. Management was escalated to therapeutic plasma exchange and cyclophosphamide. This regimen stabilized renal and hematological parameters, although severe neurological deficits and thrombocytopenia persisted. This case highlights that stroke may be a catastrophic presenting feature of AAV and that concurrent autoimmune cytopenias may add substantial diagnostic and therapeutic complexity. Early ANCA testing in cryptogenic or progressive stroke with systemic involvement is vital, and management requires careful balance between immunosuppression and infection risk.