P145 Orofacial granulomatosis in Crohn’s disease: an ECCO CONFER multi-centre case series

Abstract Background Orofacial granulomatosis (OFG) is a rare syndrome characterised by swelling of the orofacial area secondary to an underlying granulomatous inflammatory process. Concurrent intestinal Crohn’s disease (CD) has been described in 20–50% of adult patients with OFG. Methods This was a multicentre case series supported by the European Crohn’s and Colitis Organisation (ECCO) and performed as part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. A call was made to report on cases of OFG in CD, with clinical data recorded in a standardised collection form. Results This report includes 28 patients with OFG associated with CD: 14 males with mean age of 32 years ( ± 12.4 SD, range 14–60) and 14 females with mean age of 40.3 years ( ± 21.0 SD, range 11–79). Crohn’s distribution was ileal in six patients, colonic in six patients, ileocolonic in 15 patients and isolated upper gastrointestinal tract (UGI) disease in a single patient; six patients (21.4%) had UGI involvement and 11 patients (39%) had perianal disease. The diagnosis of OFG was made prior to CD diagnosis in 5 patients (mean of 0.9 years prior, range 1 month to 2 years), at the same time as CD in 4 cases, and after CD diagnosis in 19 cases (mean of 11.4 years after, range 6 months to 33 years). CD was undiagnosed in 5 patients, quiescent in 11 patients and active in 12 patients. The distribution of OFG involved the lips in 16 cases, buccal mucosa in 18 cases, tongue in eight cases and gums in five cases, with many patients having multiple areas involved. Angular cheilitis was present in 15 cases and aphthous or deep ulcers in 16 cases. Pyostomatitis vegetans and facial palsy were present in 1 case each. Pain was present in 25 cases, with impaired swallowing or speaking in 6 cases and psychological distress in 9 cases. A number of different treatments led to remission, including topical therapies in 2 cases, steroid injections in 2 cases, exclusive enteral nutrition in 1 case, anti-TNFs in 11 cases, Vedolizumab in 1 case, Ustekinumab in 1 case and Thalidomide in 2 cases. A further 7 cases were resistant to therapy including anti-TNFs and a single case had spontaneous remission without therapy. Conclusion This case series of OFG in CD highlights multiple points. The diagnosis of OFG usually occurs after that of CD and can be many decades after, but may also occur prior to or at the time of diagnosis of intestinal disease. Perianal disease and UGI disease are c