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Association between orofacial granulomatosis and Crohn's disease in children: systematic review
Marzia Lazzerini1, Matteo Bramuzzo1, Alessandro Ventura1
1Marzia Lazzerini, Matteo Bramuzzo, Alessandro Ventura, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
Insights
Orofacial granulomatosis (OFG) in children is frequently associated with Crohn's disease, with 40.4% diagnosed concurrently. OFG may represent a distinct subtype of pediatric Crohn's disease.
Area of Science:
- Pediatric Gastroenterology
- Immunodermatology
- Inflammatory Bowel Disease
Background:
- Orofacial granulomatosis (OFG) is a rare condition characterized by granulomatous inflammation of the lips, mouth, and face.
- The etiology of OFG is largely unknown, but associations with allergic reactions, infections, and autoimmune diseases have been suggested.
- A significant subset of adult OFG cases are linked to Crohn's disease (CD), a chronic inflammatory bowel disease.
Purpose of the Study:
- To systematically review pediatric cases of orofacial granulomatosis (OFG).
- To characterize the clinical presentation and disease course of OFG in children.
- To investigate the association between pediatric OFG and Crohn's disease.
Main Methods:
- Systematic review adhering to PRISMA guidelines.
- Comprehensive literature search across multiple databases (Medline, LILACS, Virtual Health Library, Web of Knowledge) up to September 2013.
- Inclusion of all pediatric OFG cases (<18 years) with full-text data extraction and manual reference searching.
Main Results:
- 173 pediatric OFG cases were identified, with a mean age of onset of 11.1 years and a 2:1 male predominance.
- Gastrointestinal symptoms were present in 26% of children at OFG diagnosis.
- A substantial 40.4% of pediatric OFG patients were diagnosed with Crohn's disease, often preceding OFG onset by an average of 13.1 months. Perianal disease and family history of CD significantly increased CD risk in OFG patients.
Conclusions:
- The high co-occurrence of Crohn's disease in children with OFG suggests a potential subtype relationship.
- OFG in pediatric populations warrants thorough evaluation for underlying inflammatory bowel disease, particularly Crohn's disease.
- Further research is needed to elucidate the shared pathophysiology between OFG and Crohn's disease.
Aim:
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.
Conclusion:
High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.
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