Pediatric granulomatosis with polyangiitis exhibiting prominent central nervous system symptoms

Granulomatosis with polyangiitis (GPA), extremely rare in children, is a disease characterized by granulomatous inflammation and necrotizing vasculitis that involves medium and small vessels. Central nervous system (CNS) involvement in GPA is not common and often appears at a later stage of the disease. The CNS manifestation of pediatric GPA was fragmentally discussed. Herein, we reported a case of relapsing-remitting pediatric GPA with prominent CNS involvement as initial symptoms.

The patient was a 9-year-old female. The first episode manifested as uroclepsia and bilateral lower extremity weakness, resulting from diffused dot-enhanced large lesions within bilateral anterior cerebral artery territory on magnetic resonance (MR). This was followed by three recurring episodes of visual impairment as a result of optic neuritis. MR in the last episode indicated bilateral sphenoid sinus inflammation adjacent to the left optical nerve. All four episodes were combined with upper respiratory tract symptoms and slight urine abnormality. She responded well to large doses of corticosteroids and immunoglobulins. However, the patient had long been suspected of having multiple sclerosis and had not received appropriate maintenance treatment.

After being diagnosed with GPA, the patient received small doses of glucocorticoids and mycophenolate mofetil for maintenance, which generated a favorable outcome.

CNS involvement in pediatric GPA is rare. Single large or multifocal insults involving one or more lobes consistent with the distribution of cerebral arteries could be a typical feature on MR. Early diagnosis and proper treatments help to improve the prognosis.