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C-ANCA/Proteinase 3–Positive Colitis in Children: A Distinctive Form of Inflammatory Bowel Disease or Vasculitis With Colitis as Initial Presentation?

Van Biervliet, Stephanie*; Bonroy, Carolien; Velde, Saskia Vande*; De Bruyne, Ruth*; Van Winckel, Myriam*; Peeters, Harald; Delanghe, Joris; Stove, Veronique; De Vos, Martine

Journal of Pediatric Gastroenterology and Nutrition: October 2013 - Volume 57 - Issue 4 - p 489–492
doi: 10.1097/MPG.0b013e31829d4e9e
Original Articles: Gastroenterology

Aim: Anti-neutrophil cytoplasmic antibodies (ANCAs) detected by indirect immunofluorescence have been found in patients with inflammatory bowel disease (IBD). Nevertheless, specific antibodies against proteinase-3 (PR3) are rare in this context.

Methods: Sera from 30 consecutive pediatric patients with IBD were evaluated for ANCA-indirect immunofluorescence and its specific antibodies to investigate whether PR3-ANCA positivity (PR3-ANCA+) identifies a distinct IBD subtype.

Results: The 5 PR3-ANCA+ patients (17%) showed significantly more concomitant biliary disease and severe anal blood loss (P < 0.05). None had vasculitis features at diagnosis nor during follow-up.

Conclusions: This pilot study demonstrates significant clinical differences between the PR3-ANCA–positive and –negative IBD subset.

*Paediatric Gastroenterology

Department of Clinical Chemistry, Microbiology and Immunology, Laboratory of Clinical Biology

Adult Gastroenterology, Ghent University Hospital, Belgium.

Address correspondence and reprint requests to Stephanie Van Biervliet, MD, PhD, Paediatric Gastroenterology and Nutrition, Ghent University Hospital (2P8), De Pintelaan 185, B-9000 Ghent, Belgium (e-mail:

Received 29 November, 2012

Accepted 11 May, 2013

Drs Van Biervliet and Bonroy contributed equally to the study.

C.B. and R.D. are supported by the Fund for Scientific Research, Flanders.

The authors report no conflicts of interest.

© 2013 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,