Extrarenal manifestations of systemic vasculitis in children

The observation data of 22 children with systemic vasculitis (SV) and morphologically verified nephritis is presented. Emphasis was put on extrarenal manifestations of SV in childhood, analysis of clinical, laboratory, immunological, morphological changes and factors influencing on the course and prognosis. It has been established that SV often manifests with general non-specific constitutional symptoms, involvement of the ENT and organs of vision, is characterized by multiple organ and generalized lesions, in 77% of cases in children is associated with anti-neutrophil cytoplasmic antibodies (ANCA). The presence of antibodies to myeloperoxidase (MPO) is noted in most patients with lesions of the skin, joints, lungs, and nervous system and is associated with an unfavorable renal prognosis. ENT and organs of vision, gastrointestinal tract involvement is equally often observed in both MPO and PR3 antibody positive.