title: pANCA and ASCA in Children with IBD-Unclassified, Crohn's Colitis, and Ulcerative Colitis-A Longitudinal Report from the IBD Porto Group of ESPGHAN.
abstract: No study to date has evaluated perinuclear antineutrophil cytoplasmic antibody (pANCA) and anti-Saccharomyces cerevisiae antibody (ASCA) in pediatric inflammatory bowel disease-unclassified (IBDU) as compared with Crohn's colitis (CC) and ulcerative colitis (UC), which represent the diagnostic challenge. We aimed to explore the diagnostic utility of serology and to assess whether serology can predict disease severity in these subgroups.
  
  
  
  This was a multicenter retrospective longitudinal study including 406 children with inflammatory bowel diseases (IBD) from 23 centers affiliated with the Porto group of European Society of Pediatric Gastroenterology, Hepatology and Nutrition (mean age 10.5 ± 3.9, 54% males); 117 (29%) with CC, 143 (35%) with UC, and 146 (36%) with IBDU. Median follow-up period was 2.8 years (interquartile range, 1.6-4.2).
  
  
  
  The most prevalent serologic profile in IBDU was pANCA-/ASCA- (41%), followed by pANCA+/ASCA- (34%) and pANCA-/ASCA+ (17%). pANCA-/ASCA+ differentiated well between CC versus IBDU (83% specificity, 96% positive predictive value [PPV]) and UC (97% specificity, 90% PPV) patients, albeit with a low negative predictive value (13% and 40%, respectively). pANCA+/ASCA- did not differentiate as well between IBD subgroups, but UC children with pANCA+/ASCA- had more often severe disease at diagnosis (36 [62%] versus 22 [38%], P = 0.033) and needed more often calcineurin inhibitors, biologics, or colectomy (25 [80%] versus 6 [20%], P = 0.026). In CC, double positivity for ASCA and not pANCA-/ASCA+ profile was associated with disease severity.
  
  
  
  Serology may have some role in predicting disease course and outcomes in colonic IBD, but its routine use needs to be supported by more studies. Serology cannot routinely be recommended for differentiating between IBDU versus CC or UC as a sole diagnostic criterion given its low diagnostic utility.
authors.affiliation: 1Shaare Zedek Medical Center, Jerusalem, Israel;2Child Life and Health, University of Edinburgh, United Kingdom;3University of Helsinki, Finland;4Department of Pediatric Gastroenterology, Medical University, Warsaw, Poland;5University Hospital Southampton, United Kingdom;6University Hospitals Bristol, Scotland, United Kingdom;7University of Messina, Italy;8University of Florence, Italy;9University Hospital for Pediatrics and Adolescent Medicine of the Medical University of Graz, Austria;10Department of Pediatric Gastroenterology and Nutrition, Robert Debré Hospital, Paris, France;11Semmelweis University, Budapest, Hungary;12Erasmus MC, Rotterdam, Zuid Holland, Netherlands; and13The Hebrew University of Jerusalem, Israel.
authors.forename: Liron
authors.initials: L
authors.lastname: Birimberg-Schwartz
authors.forename: David C
authors.initials: DC
authors.lastname: Wilson
authors.forename: Kaija-Leena
authors.initials: KL
authors.lastname: Kolho
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Karolewska-Bochenek
authors.forename: Nadeem Ahmad
authors.initials: NA
authors.lastname: Afzal
authors.forename: Christine
authors.initials: C
authors.lastname: Spray
authors.forename: Claudio
authors.initials: C
authors.lastname: Romano
authors.forename: Paolo
authors.initials: P
authors.lastname: Lionetti
authors.forename: Almuthe C
authors.initials: AC
authors.lastname: Hauer
authors.forename: Christine
authors.initials: C
authors.lastname: Martinez-Vinson
authors.forename: Gabor
authors.initials: G
authors.lastname: Veres
authors.forename: Johanna C
authors.initials: JC
authors.lastname: Escher
authors.forename: Dan
authors.initials: D
authors.lastname: Turner
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D000907:Antibodies, Bacterial / Q000097:blood*; D001688:Biological Products / Q000627:therapeutic use; D065095:Calcineurin Inhibitors / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003093:Colitis, Ulcerative / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D003424:Crohn Disease / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000097:blood / Q000175:diagnosis; D008137:Longitudinal Studies; D008297:Male; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012441:Saccharomyces cerevisiae / Q000276:immunology*; D012720:Severity of Illness Index
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000907:Antibodies, Bacterial; D001688:Biological Products; D065095:Calcineurin Inhibitors