Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients☆,☆☆,★
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)
- et al.
Anti-neutrophil cytoplasmic autoantibodies and associated diseases: a review
Am J Kidney Dis
(1990) - et al.
Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential
Kidney Int
(1994) - et al.
Antineutrophil cytoplasm antibody in crescentic glomerulonephritis
Arch Dis Child
(1988) - et al.
Anti-neutrophil cytoplasmic autoantibodies in a child with pauci-immune necrotizing and crescentic glomerulonephritis
Pediatr Nephrol
(1991)
Cited by (42)
Antineutrophil cytoplasmic autoantibodies in children: Clinical features
2013, Archives de PediatrieGlomerulotubular Dysfunction and Acute Kidney Injury
2011, Pediatric Critical Care: Expert Consult Premium EditionGlomerulotubular Dysfunction and Acute Kidney Injury
2011, Pediatric Critical CarePulmonary Manifestations of Systemic Vasculitis
2009, Pulmonary Manifestations of Pediatric DiseasesGlomerulotubular Dysfunction and Acute Renal Failure
2006, Pediatric Critical CareClinical and biological symptoms of glomerular diseases
2005, EMC - Pediatrie
- ☆
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