Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients,☆☆,

https://doi.org/10.1016/S0022-3476(95)70497-3Get rights and content

Abstract

Anti-neutrophil cytoplasmic autoantibodies (ANCA) are usually determined during the diagnostic evaluation of systemic vasculitis and glomerulonephritis syndromes in adult patients, but few pediatric patients with ANCA have been reported. We describe five pediatric patients with ANCA and glomerulonephritis, with and without upper or lower respiratory tract disease. We compared these five patients and six previously described patients to affected adults; the spectrum of ANCA-associated disease appears to be similar in adults and children, but a female predominance may exist in the pediatric patients. Pediatric patients often had end-stage renal disease within 1 year after onset. We conclude that ANCA is a useful diagnostic tool in both pediatric and adult patients with systemic vasculitis and glomerulonephritis. (J PEDIATR 1995;126: 40-3)

Section snippets

CASE REPORTS

The clinical characteristics of our five patients are listed in Table I.

METHODS

In all patients except No. 3, ANCA were detected by IIF microscopy and by ELISA in the nephropathology laboratory at the University of North Carolina (J. Charles Jennette, director, Chapel Hill, N.C.); normal values on the ELISA are < 40. In patient 3, ANCA determinations were done by flow cytometry and IIF antibody microscopy at Specialty Laboratories Inc. (Santa Monica, Calif.); the normal value for flow cytometry is < 22. The pattern of ANCA (C- or P-ANCA) was determined by IIF microscopy.

We

RESULTS

All pediatric patients in our study had a prodrome lasting several weeks to months, which often consisted of abdominal pain, fever, or weight loss. Of the 11 patients, 9 were female; the age at onset averaged 11.5 ± 2.5 years and was most often the early adolescent years. Although race is not listed in the previously reported cases, all five of our patients were white, a disproportionate number in view of the racial makeup of our population base. Seven of the pediatric patients had C-ANCA

DISCUSSION

Our five patients have a spectrum of ANCA-associated disease from renal-limited disease to renal disease with other systemic symptoms or pulmonary vasculitis. In addition, one of our patients had recurrent crescentic glomerulonephritis in a cadaveric renal allograft, which resulted in loss of transplant function; this same patient had onset of pulmonary symptoms with pulmonary vasculitis and hemorrhage 6 years after the onset of renal disease.

Of adult patients in several reported series,6, 7, 8

References (8)

There are more references available in the full text version of this article.

Cited by (42)

  • Glomerulotubular Dysfunction and Acute Kidney Injury

    2011, Pediatric Critical Care: Expert Consult Premium Edition
  • Pulmonary Manifestations of Systemic Vasculitis

    2009, Pulmonary Manifestations of Pediatric Diseases
View all citing articles on Scopus

From the Department of Pediatrics, Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, and Department of Pediatrics, Cardinal Glennon Children's Hospital and St. Louis University Health Sciences Center, St. Louis, Missouri.

☆☆

Reprint requests: Eileen N. Ellis, MD, Pediatric Nephrology, Arkansas Children's Hospital, 800 Marshall, Little Rock, AR 72202.

0022-3476/95/$3.00 + 0 9/20/59590

View full text