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Portuguese Journal of Nephrology & Hypertension

Print version ISSN 0872-0169


RODRIGUES, Sara Daniela et al. Cerebral vasculitis - a rare presentation of antineutrophil cytoplasmic antibody associated vasculitis relapse. Port J Nephrol Hypert [online]. 2019, vol.33, n.1, pp.47-50. ISSN 0872-0169.

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis commonly involves kidney, lung, upper respiratory tract, skin, gastrointestinal and occasionally peripheral nervous system. Central nervous system is less commonly affected and is generally part of a multi-organ scenario. We present a case of a 21-year-old female with chronic kidney disease secondary to myeloperoxidase ANCA associated vasculitis, diagnosed at age 19. She then presented with pulmonary-renal syndrome with stage 5 CKD. Three months’ cyclophosphamide and prednisone were instituted, followed by azathioprine, but she remained dialysis dependent. After 2 years, while on maintenance treatment, she was admitted to the emergency department with tonic-clonic seizures. She had started oral ciprofloxacin 2 days before for acute gastroenteritis. No illicit drugs were noticed. Blood tests showed an increased myoglobin but were otherwise unremarkable. No anomalies were reported on cerebral computed tomography or cerebrospinal fluid analysis. Magnetic resonance imaging showed diffuse cortico-subcortical lesions with T2 and proton density hypersignal suggesting recent ischemia, and segmental irregularities of various cerebral and vertebral arteries, consistent with small and medium size vessel vasculitis. Intravenous methylprednisolone and oral cyclophosphamide were started. Anti-myeloperoxidase ANCA levels were increased (>200 RU/mL), reinforcing our diagnostic hypothesis of central nervous system (CNS) recurrence of ANCA vasculitis. The patient had a favorable clinical course, with no neurological sequelae. Conclusions: This is a case of ANCA associated vasculitis that presented with kidney and lung but no CNS involvement and recurred with primarily cerebral disease. Favorable outcome was seen with standard immunosuppression. Awareness of this rare but severe complication is critical for timely recognition and prompt treatment.

Keywords : ANCA; antineutrophil cytoplasmic antibody; Central Nervous System Vasculitis; relapse; vasculitis.

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