Elsevier

The Journal of Pediatrics

Volume 132, Issue 2, February 1998, Pages 325-328
The Journal of Pediatrics

Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: A single-center experience,☆☆,

https://doi.org/10.1016/S0022-3476(98)70453-6Get rights and content

Abstract

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 with ANCA-associated glomerulonephritis progress to end-stage renal disease. Seven children (13.0 ± 0.89 years, mean age ± SEM) with both a focal and necrotizing glomerulonephritis and a positive ANCA titer are described. Presenting symptoms were constitutional (100%) and sinopulmonary (71%); additional renal features included microscopic hematuria (100%), proteinuria (71%), and renal insufficiency (71%). Acute therapy (0 to 2 weeks from diagnosis) included intravenous corticosteroids and intravenous cyclophosphamide for all patients. Induction therapy (2 weeks to 6 months from diagnosis) consisted of cyclophosphamide (100%) and daily corticosteroids (86%) for a minimum of 6 months. Maintenance 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 ± 12 months in all seven patients revealed that only one (14%) patient had end-stage renal disease, whereas the remaining patients had microscopic hematuria (100%), proteinuria (50%), and renal insufficiency (33%). These findings suggest that early recognition and aggressive treatment of children with ANCA-associated glomerulonephritis and vasculitis may result in an improved renal outcome compared with previous reports. (J Pediatr 1998;132:325-8)

Section snippets

Patient Selection

A retrospective analysis of all children presenting to the University of Michigan Medical Center between July 1989 and November 1996 with a focal and necrotizing glomerulonephritis and a positive ANCA titer was performed. Seven patients met these criteria. Data are presented as the mean ± SEM.

Glomerulonephritis was defined as the presence of hematuria (250/ μl), proteinuria (≥100 mg/dl or ≥2+), or both on urinalysis with or without renal insufficiency or hypertension. Blood pressure,

Presentation

The mean age of the seven affected children was 13.0 ± 0.9 years (range 11 to 17 years). Five (71%) patients were female. Four (57%) patients were cANCA-positive, and three (43%) were pANCA-positive. Features at presentation included constitutional symptoms (100%), sinopulmonary symptoms (71% [5 of 7]), and arthralgias (57% [4 of 7]). Microscopic hematuria was present in 100% of patients; ≥2+ proteinuria and renal insufficiency were seen in 71% (5 of 7) of patients. Two patients had microscopic

DISCUSSION

The morbidity of pediatric ANCA-associated glomerulonephritis (13 patients treated at six different centers) is high.8, 9, 10, 11, 12 Renal insufficiency at presentation is an important determinant of long-term renal outcome. The mean presenting serum creatinine in our patients (2.9 ± 0.9 mg/dl [range 0.7 to 6.9 mg/dl]) was much better than that in the studies with poor renal outcomes (10.1 and 10.5 mg/dl, respectively.8, 11 This better renal function at presentation suggests that our patients

Acknowledgements

The authors thank W. Joseph McCune, MD, for his helpful discussion regarding the treatment of these children with ANCA-associated glomerulonephritis and vasculitis.

Cited by (23)

  • Glomerulonephritis in Children

    2010, Pediatric Urology
  • Glomerulonephritis in children

    2009, Pediatric Urology: Expert Consult
  • Antineutrophil cytoplasmic antibodies and associated diseases: A review of the clinical and laboratory features

    2000, Kidney International
    Citation Excerpt :

    However, in children over the age of 7 and in adolescents, the features resemble adult disease, except that subglottic stenosis and nasal deformity are more common, and fewer cyclophosphamide-related malignancies ensue72. As in adults, early recognition and treatment are important to improve the renal outcome73. Pregnancy is uncommon in the age group affected by Wegener's granulomatosis, but may trigger the onset of disease and relapse74.

  • Glomerulonephritis

    2005, Adolescent Medicine Clinics
View all citing articles on Scopus

From the Division of Pediatric Nephrology, Department of Pediatrics, University of Michigan, Ann Arbor, and the Department of Pediatrics, University of Rochester, Rochester, New York.

☆☆

Reprint requests: Rudolph P. Valentini, MD, Children's Hospital of Michigan, Division of Nephrology, 3901 Beaubien Blvd., Detroit, MI 48201-2196.

0022-3476/98/$5.00 + 0  9/21/83689

View full text