ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features-outcome.
BACKGROUND
Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and systemic vasculitis (AAGNV) is uncommon in childhood.


METHODS
This is a retrospective study of AAGNV cases diagnosed over a 13-year period in a tertiary pediatric nephrology department.


RESULTS
Thirteen cases of AAGNV were identified: seven Wegener granulomatosis (WG) and six microscopic polyangiitis (MPA). Acute renal failure/nephrotic range proteinuria (NRP) was found in 77 % of the patients (4 with WG, all with MPA). Eleven (85 %) patients showed necrotizing glomerulonephritis (NGN), with ≥50 % crescents identified in nine patients (69 %) (4 with WG, 5 with MPA). Treatment with methylprednisolone, cyclophosphamide and plasma exchange resulted in extra-renal remission and antibody reduction in all patients and renal function improvement/stabilization in 77 % of the patients. Three patients, all without oliguria at presentation and few sclerotic lesions, had normal renal function at follow-up. Chronic kidney disease (CKD) stages 2 and 3-4 were observed in four (WG) and three (MPA) patients, respectively. Three patients (23 %) developed end stage renal disease: two were MPA patients with severe presentation (markedly impaired glomerular filtration rate, oliguria, NRP, crescentic NGN, glomerular sclerosis) and one was a WG patient with extensive interstitial fibrosis/tubular atrophy.


CONCLUSIONS
Severe renal involvement was more common in children with MPA than WG. Treatment with methylprednisolone, cyclophosphamide and plasma exchange induced extra-renal remission/serological response and renal function improvement/stabilization. Markedly decreased GFR, oliguria, NRP, and chronic glomerular lesions at presentation were predictors of poor outcome.
Department of Nephrology, Birmingham Children's Hospital NHS Foundation Trust, Steelhouse Lane, Birmingham, B4 6NH, UK. eksiomou@yahoo.gr
Ekaterini
E
Siomou
Despoina
D
Tramma
Claire
C
Bowen
David V
DV
Milford
D058186:Acute Kidney Injury / Q000276:immunology; D000293:Adolescent; D000367:Age Factors; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004359:Drug Therapy, Combination; D004739:England; D005260:Female; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D005938:Glucocorticoids / Q000008:administration & dosage; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007668:Kidney / Q000187:drug effects / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000276:immunology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D055953:Microscopic Polyangiitis / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D010951:Plasma Exchange; D011507:Proteinuria / Q000276:immunology; D020551:Pulse Therapy, Drug; D051436:Renal Insufficiency, Chronic / Q000276:immunology; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone