Abstract Background Orofacial granulomatosis (OFG) is an uncommon, chronic inflammatory disease characterised by relapsing and remitting lip swelling and ulceration of the buccal mucosa, gingiva and floor of the mouth. The histology is of non-caseating granulomas which are indistinguishable from those seen in Crohn’s disease (CD) and there is overlap with oral CD. There is a strong association between CD and OFG, with studies suggesting that up to 1/3 of patients with OFG either have or will develop CD. There is limited data on the phenotype of CD seen in association with OFG. A multicentre case series supported by ECCO suggested possible high rates of UGI and perianal CD (pCD) but the patient number was small (n = 28) 1. Methods The aim of the study was to characterise the phenotype of CD in a large cohort of patients with both CD and OFG using the Montreal classification. Patients with OFG and CD were identified from the databases of two UK tertiary referral centres for IBD. The case notes were reviewed and the pattern of disease classified. The surgical history and treatment history was also recorded. Results 132 patients were identified with CD and OFG. Clinical data was available for 109 patients. 49 were female and the median age was 42 (IQR 34-57). Median age at diagnosis was 20 (IQR 15-29) and median disease duration was 21 years (14-29). The Montreal classification (age at diagnosis, disease distribution and disease behaviour) is described in table 1. The prevalence of perianal disease (pCD) was 46% (50/109). 44 patients had undergone gastroscopy with 16 (15%) having findings thought to be consistent with upper gastrointestinal CD (UGI CD). Of these 16, 13 (12%) had oesophageal involvement with 10 (9%) having stricturing oesophageal disease. 82 (75%) patients received advanced therapy and 18 (17%) azathioprine alone. The median number of advanced therapies was one with anti-TNF being the most common (79 patients (72%)). 44 (40%) patients had undergone resectional surgery. Conclusion To our knowledge, this is the largest study to date of the phenotypic pattern of CD in patients with OFG and CD. Our study demonstrates that, compared to other published datasets for patients with CD alone, patients with OFG and CD are diagnosed at a young age (20 vs 29.52) and there are high rates of pCD (46% vs 20%3), (although acknowledging that this is an uncontrolled comparison). UGI involvement is relatively common, with high rates of oesophageal involvement (12% vs 4%4) and particularly oesophageal stricturing disease. Our results suggest that clinicians should have a low threshold for performing UGI endoscopy in all patients with CD/OFG and the high rates of UGI and pCD suggests that early escalation to advanced therapies is appropriate. References: 1. Phillips F, Verstockt B, Sladek M et al. J Crohns Colitis 2022;16(3):430-435 2. Molodecky NA, Soon IS, Rabi DM et al Gastroenterology 2012;142(1):46-54 3. Tsai L, McCurdy JC, Ma C et al Inflamm Bowel Disease 2022;28(10):1477-843. 4. De Felice KM, Katzka DA, Raffals LE. Inflamm Bowel Disease 2015;21(9):2106-2113 Conflict of interest: Lees, Robert: No conflict of interest Molinillo Marin, Angelica: No conflict of interest Patel, Priyanka: No conflict of interest Harte, Molly: No conflict of interest Ormond, Martyn: No conflict of interest Herbert, Cameron: No conflict of interest Hullah, Esther: No conflict of interest Lamb, Christopher Andrew: Grant: In the last 5 years I have undertaken research supported by grants from the following: Genentech, Janssen, Takeda, AbbVie, Eli Lilly, Pfizer, Roche, UCB Biopharma, Sanofi Aventis, Biogen IDEC, Orion OYJ and AstraZeneca. Personal Fees: In the last 5 years I have received honoraria for speaking at educational events from Takeda, Janssen, Dr Falk, Ferring and Nordic pharma. Other: The following companies were corporate sponsors of an educational event, IBD Newcastle 2023, I convened at Newcastle University on 29th November 2023 Amgen, Celltrion Healthcare, Janssen, Tillotts Pharma, Pharmacosmos, Dr Falk Pharma, Galapagos, Ferring, AbbVie, Takeda, Eli Lilly and Bristol Myers Squibb. Speight, Ally: No conflict of interest Dr. Mawdsley, Joel: No conflict of interest
Lees et al. (Thu,) studied this question.