Author(s)

Christine Sethna, Rachel Frank, Lulette Infante, Beth Gottlieb, Anne Eberhard, Xiaotong Wang, Howard Trachtman

Department of Pathology, North Shore University Hospital of the North Shore—LIJ Health System, New York, USA.
Department of Pediatrics, Division of Nephrology, Cohen Children’s Medical Center of New York, New York, USA.
Department of Pediatrics, Division of Nephrology, NYU Langone Medical Center, New York, USA.
Department of Pediatrics, Division of Rheumatology, Cohen Children’s Medical Center of New York, New York, USA.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.

Anti-Neutrophil Cytoplasmic Antibody (ANCA); Vasculitis; Epidemiology

C. Sethna, R. Frank, L. Infante, B. Gottlieb, A. Eberhard, X. Wang and H. Trachtman, "Anti-Neutrophil Cytoplasmic Antibody Vasculitis in Pediatric Patients: Is the Incidence Rising?," Open Journal of Nephrology, Vol. 2 No. 2, 2012, pp. 19-22. doi: 10.4236/ojneph.2012.22003.