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Pediatric patients with orofacial granulomatosis likely to subsequently develop intestinal Crohn's disease: Brief
Kristen L Chen1, Daren A Diiorio2, Yvonne E Chiu3
1School of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Insights
Orofacial granulomatosis (OFG) may indicate underlying Crohn's disease. Many OFG patients later develop intestinal Crohn's, necessitating long-term gastroenterology monitoring for early diagnosis.
Area of Science:
- Pediatric Gastroenterology
- Dermatology
- Immunology
Background:
- Orofacial granulomatosis (OFG) is a rare chronic inflammatory condition affecting the face and lips.
- The relationship between OFG and Crohn's disease (CD) remains debated, with OFG potentially being a distinct entity or an oral manifestation of CD.
Purpose of the Study:
- To investigate the association between orofacial granulomatosis and the subsequent development of Crohn's disease in pediatric patients.
- To determine the diagnostic yield of monitoring OFG patients for intestinal CD.
Main Methods:
- Retrospective review of 7 pediatric patients diagnosed with OFG between 2000 and 2018.
- Longitudinal follow-up to identify subsequent diagnoses of Crohn's disease.
Main Results:
- Four out of seven OFG patients (57%) were diagnosed with Crohn's disease.
- The median time from OFG diagnosis to CD diagnosis was 3.1 years (range 0.4–6.9 years).
Conclusions:
- Orofacial granulomatosis may serve as an early indicator of underlying Crohn's disease in pediatric patients.
- Gastroenterology evaluation and sustained monitoring are crucial for early detection of intestinal Crohn's disease in individuals with OFG.
Abstract:
Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition presenting as perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn's disease. Our retrospective review identified 7 patients diagnosed with OFG between 2000 and 2018 at a tertiary pediatric hospital. Four of the 7 patients subsequently developed Crohn's disease with a median delay of 3.1 years (range 0.4-6.9 years). This indicates that gastroenterology evaluation with long-term monitoring for intestinal Crohn's disease is warranted.
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