Related Experiment Video
Updated: Sep 8, 2025
Anti-Nuclear Antibody Screening Using HEp-2 Cells
Published on: June 23, 2014
Anti-Proteinase 3 Antibodies as a Biomarker for Ulcerative Colitis and Primary Sclerosing Cholangitis in Children
Martin Walter Laass1, Josefine Ziesmann1,2,3, Jan de Laffolie4
1Department of Paediatrics, University Hospital and Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Insights
Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) may serve as a marker in pediatric inflammatory bowel disease (IBD). PR3-ANCA was prevalent in ulcerative colitis (UC) and associated with primary sclerosing cholangitis (PSC).
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Serology
Background:
- Anti-neutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis.
- ANCA and PR3-ANCA are also observed in patients with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC).
Purpose of the Study:
- To investigate the prevalence and significance of ANCA and PR3-ANCA in pediatric IBD patients.
- To explore the association of PR3-ANCA with disease characteristics and liver manifestations in pediatric IBD.
Main Methods:
- Tested 326 pediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence), and PR3-ANCA (chemiluminescence immunoassay).
- Applied the Paris classification for pediatric IBD and documented liver manifestations like primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH).
Main Results:
- PR3-ANCA was found in 40% of UC, 11% of Crohn disease (CD), and 11% of IBD-unclassified (IBD-U) patients, but not in controls.
- PR3-ANCA positivity in UC correlated with more extensive disease and was frequent in UC patients with backwash ileitis (67%).
- PR3-ANCA was detected in 68% of patients with PSC or PSC/AIH, significantly higher than in those without liver disease (32%), and associated with elevated liver enzymes.
Conclusions:
- PR3-ANCA demonstrates potential as a serological marker for pediatric ulcerative colitis.
- PR3-ANCA may also serve as a marker for primary sclerosing cholangitis in pediatric IBD patients.
Objectives:
Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis, but is also found in patients with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). The aim of our study was to investigate ANCA and PR3-ANCA in paediatric IBD.
Methods:
We tested 326 paediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence, IIF) and PR3-ANCA (chemiluminescence immunoassay). We applied the Paris classification for paediatric IBD and documented liver manifestations such as primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH).
Results:
We found PR3-ANCA in 49/121 (40%) of UC, 20/187 (11%) of Crohn disease (CD) and 2/18 (11%) of IBD-unclassified (IBD-U) patients but in none of the controls. 54% UC and 12% CD patients were positive for ANCA (IIF). PR3-ANCA positive UC patients were characterised by more extensive disease (P = .070). Fourteen of 21 (67%) of UC patients with backwash ileitis were anti-PR3 ANCA-positive (P = .011). We diagnosed PSC or PSC/AIH in 19 UC and 3 IBD-U patients. Fifteen of 22 (68%) patients with PSC or PSC/AIH were anti-PR3-ANCA positive in contrast to 36 of 117 (32%) patients without PSC (P = .001). PR3-ANCA positive patients showed higher levels of gamma-glutamyl transferase, alanine transaminase and aspartate transferase (P < 0.001, 0.001, 0.006, respectively). Forty-seven percent of CD and 6% of UC patients were ASCA-IgA positive. PR3-ANCA-positive and -negative patients showed no significant differences concerning age at diagnosis, disease activity, need for drugs, and number of hospitalisations.
Conclusions:
Our study provides data for PR3-ANCA as a potential serological marker for paediatric UC and PSC.
More Related Videos
06:50A High-Throughput Electrochemiluminescence 7-Plex Assay Simultaneously Screening for Type 1 Diabetes and Multiple Autoimmune Diseases
Published on: May 29, 2020
05:45Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Related Concept Videos
01:28Peptic Ulcer Disease III: Clinical Manifestations and Diagnostic Studies
Few clinical manifestations differentiate gastric ulcers from duodenal ulcers. Distinctions in the location, timing, and pain relief are crucial for healthcare providers in differentiating between gastric and duodenal ulcers during clinical assessments.
01:30Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy
Diagnostic studies
A colonoscopy is the definitive screening test, distinguishing ulcerative colitis from other colon diseases with similar symptoms. During a colonoscopy test, inflamed mucosa with exudate ulcerations can be observed, and biopsies are taken to determine the histologic characteristics of the...
01:17Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...