OUTCOME OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES-POSITIVE GLOMERULONEPHRITIS AND VASCULITIS IN CHILDREN - A SINGLE-CENTER EXPERIENCE

Vasculitis associated with antineutrophil cytoplasmic autoantibodies ( ANCA) can be accompanied by a focal and necrotizing glomerulonephritis that carries a high morbidity. As many as 60% of reported children wi th ANCA-associated glomerulonephritis progress to end-stage renal dise ase. Seven children (13.0 +/- 0.89 years, mean age +/- SEM) with both a focal and necrotizing glomerulonephritis and a positive ANCA titer a re described. Presenting symptoms were constitutional (100%) and sinop ulmonary (71%); additional renal features included microscopic hematur ia (100%), proteinuria (71%), and renal insufficiency (71%). Acute the rapy (0 to 2 weeks from diagnosis) included intravenous corticosteroid s and intravenous cyclophosphamide for all patients. Induction therapy (2 weeks to 6 months from diagnosis) consisted of cyclophosphamide (1 00%) and daily Corticosteroids (86%) for a minimum of 6 months. Mainte nance therapy that followed 6 months of induction therapy consisted of alternate day steroids (100%) combined with either oral azathioprine (50%) or oral cyclophosphamide (50%). Long-term follow-up for 48 +/- 1 2 months in all seven patients revealed that only one (14%) patient ha d end-stage renal disease, whereas the remaining patients had microsco pic hematuria (100%), proteinuria (50%), and renal insufficiency (33%) . These findings suggest that early recognition and aggressive treatme nt of children with ANCA-associated glomerulonephritis and vasculitis may result in an improved renal outcome compared with previous reports .