title: Does Crohn's Disease with Concomitant Orofacial Granulomatosis Represent a Distinctive Disease Subtype?
abstract: BACKGROUND
  Although orofacial granulomatosis (OFG) may present as a separate clinical entity, it often seems in conjunction with various systemic diseases, of which Crohn's disease (CD) is one of the most common. The aim of this study was to investigate whether CD with concomitant OFG represents a distinctive disease subtype.
  
  
  METHODS
  Twenty-one patients with CD and concomitant OFG (CD+OFG group) were included in the study. As the reference group, a cohort of 39 patients with CD but without OFG (CD-R group) was used. Demographic data and clinical characteristics were recorded at the time of diagnosis. The 2 groups were compared using multivariate analyses.
  
  
  RESULTS
  The percentage of patients with intestinal inflammation in the upper gastrointestinal tract was significantly higher in the CD+OFG group, as compared with the CD-R group (81% versus 33%; P < 0.001). Furthermore, ileocolonic inflammation was significantly more common in the CD+OFG patients (81% versus 46%; P = 0.013). In addition, perianal disease was more frequently observed in the CD+OFG group (48% versus 18%; P = 0.033). Significantly more patients showed evidence of granulomas in the primary endoscopy in the CD+OFG group than in the CD-R group (81% versus 38%; P = 0.003).
  
  
  CONCLUSIONS
  The data from this study suggest that the presence of CD in conjunction with OFG represents a distinctive subphenotype of CD that is characterized by extensive inflammation, perianal disease, and pronounced granuloma formation in the intestine.
authors.affiliation: *Department of Oral Medicine and Pathology, Institute of Odontology, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; †Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; ‡Department of Infectious Medicine, Institute of Biomedicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; §Department of Women's and Children's Health, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Karolinska Institute, Stockholm, Sweden; and ‖Paediatric Dentistry, Eastman Institute, Public Dental Health, Stockholm, Sweden.
authors.forename: Gita
authors.initials: G
authors.lastname: Gale
authors.forename: Gudmundur Vignir
authors.initials: GV
authors.lastname: Sigurdsson
authors.forename: Sofia
authors.initials: S
authors.lastname: Östman
authors.forename: Petter
authors.initials: P
authors.lastname: Malmborg
authors.forename: Karin
authors.initials: K
authors.lastname: Högkil
authors.forename: Bengt
authors.initials: B
authors.lastname: Hasséus
authors.forename: Mats
authors.initials: M
authors.lastname: Jontell
authors.forename: Robert
authors.initials: R
authors.lastname: Saalman
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003424:Crohn Disease / Q000145:classification* / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D051261:Granulomatosis, Orofacial / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008297:Male; D011379:Prognosis; D055815:Young Adult