Abstract
We present a very rare case of systemic disease p-ANCA positive necrotizing vasculitis in an 11-year-old child. Polyangiitis microscopica belongs to the group of ANCA vasculitis.
The disease is characterized beside presence of non-specific symptoms with typical kidney involvement based on characteristic histological finding. Coincident lung and kidney involvement, the so called pulmorenal syndrome has much more poor prognosis.
The most common treatment of progressive forms of the disease is based on immunosuppressants: corticosteroids (prednisolon, methylprednisolon) in combination with cytotoxic drugs (cyclophosphamide, azathioprine). Plasmapheresis is indicated in an acute progressive phase of the disease resistant to the immunosuppression and in case of pulmorenal syndrome.
Combination of rituximab+corticosteroids has been proved by FDA in 2011 in the treatment in adulthood. The treatment should be long-lasting because of frequent relapses of the disease.