Patient:
Female, 10 FINAL DIAGNOSIS: Polyangiitis Symptoms: Intracranial hemorrhage • swelling • oliguria
Medication:
Cyclophosphamide • prednisolone Clinical Procedure: Plasmapheresis Specialty: Paediatric nephrology • nephrology • paediatrics.
Objective:
Rare disease.
Background:
MPO ANCA-associated vasculitis is very rare in children. Renal disease is almost universally present but lung and central nervous system involvement are not commonly reported.
Case report:
We present a pediatric case of microscopic polyangiitis with the unusual presentation of pauci-immune glomerulonephritis, intracerebral hemorrhage and pulmonary hemorrhage. The neurological and pulmonary symptoms settled after treatment with cyclophosphamide and plasmapheresis. However, there was no renal recovery and the patient was rendered dialysis-dependent.
Conclusions:
We believe that this is the first reported case of pediatric microscopic polyangiitis with central nervous system involvement. The disease may have a subclinical presentation resulting in chronic damage to different organs. Prompt treatment of acute disease with immunosuppression and plasmapheresis can halt disease progression in these patients.