Oro-facial granulomatosis: Crohn's disease or a new inflammatory bowel disease?

Inflamm Bowel Dis. 2005 Sep;11(9):840-6. doi: 10.1097/01.mib.0000178261.88356.67.

Abstract

Background: Oro-facial granulomatosis (OFG) is a rare chronic inflammatory disorder presenting characteristically with lip swelling but also affecting gingivae, buccal mucosa, floor of mouth, and a number of other sites in the oral cavity. Histologically, OFG resembles Crohn's disease (CD), and a number of patients with CD have oral involvement identical to OFG. However, the exact relationship between OFG and CD remains unknown.

Methods: Thirty-five patients with OFG and no gut symptoms were identified from a combined oral medicine/gastroenterology clinic. All underwent a standardized assessment of the oral cavity and oral mucosal biopsy to characterize the number of sites affected and the type of inflammation involved. Hematological and biochemical parameters were also recorded. All 35 patients underwent ileocolonoscopy and biopsy to assess the presence of coexistent intestinal inflammation.

Results: Ileal or colonic abnormalities were detected in 19/35 (54%) cases. From gut biopsies, granulomas were present in 13/19 cases (64%). An intestinal abnormality was significantly more likely if the age of OFG onset was less than 30 years (P=0.01). Those with more severe oral inflammation were also more likely to have intestinal inflammation (P=0.025), and there was also a correlation between the histologic severity of oral inflammation and the histologic severity of gut inflammation (P=0.047). No relationship was found between any blood parameter and intestinal involvement.

Conclusions: Endoscopic and histologic intestinal abnormalities are common in patients with OFG with no gastrointestinal symptoms. Younger patients with OFG are more likely to have concomitant intestinal involvement. In these patients, granulomas are more frequent in endoscopic biopsies than reported in patients with documented CD. OFG with associated intestinal inflammation may represent a separate entity in which granulomatous inflammation occurs throughout the gastrointestinal tract in response to an unknown antigen or antigens.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Antigens / immunology
  • Biopsy
  • Child
  • Colonoscopy
  • Crohn Disease / complications*
  • Diagnosis, Differential
  • Female
  • Granulomatosis, Orofacial / etiology
  • Granulomatosis, Orofacial / immunology*
  • Granulomatosis, Orofacial / pathology*
  • Humans
  • Inflammation
  • Intestines / immunology
  • Intestines / pathology
  • Male
  • Middle Aged

Substances

  • Antigens