title: Association between orofacial granulomatosis and Crohn's disease in children: systematic review.
abstract: OBJECTIVE
  To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease.
  
  
  METHODS
  We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (< 18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients' characteristics, disease characteristics, association with other diseases, and treatment. We analyzed the data and reported the results in tables and text.
  
  
  RESULTS
  We retrieved 173 reports of OFG in children. Mean age at onset was 11.1 ± 3.8 years (range: 2.0-18 years). Prevalence in males was significant higher than in females (P < 0.001), with a male:female ratio of 2:1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnosis. Overall, 70/173 (40.4%) children received a concomitant diagnosis of Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P < 0.0001 for both). Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab.
  
  
  CONCLUSIONS
  High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.
authors: Marzia Lazzerini, Matteo Bramuzzo, Alessandro Ventura, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
authors: Marzia
authors: M
authors: Lazzerini
authors: Marzia Lazzerini, Matteo Bramuzzo, Alessandro Ventura, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
authors: Matteo
authors: M
authors: Bramuzzo
authors: Marzia Lazzerini, Matteo Bramuzzo, Alessandro Ventura, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
authors: Alessandro
authors: A
authors: Ventura
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003424:Crohn Disease / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D051261:Granulomatosis, Orofacial / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D015995:Prevalence; D012307:Risk Factors; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids
keywords: Cheilitis granulomatosa; Children; Crohn’s disease; Melkersson-Rosenthal syndrome; Orofacial granulomatosis; Systematic review