{
  "abstract": "Introduction Orofacial granulomatosis (OFG) is a rare chronic inflammatory condition of the face and mouth, which is strongly associated with Crohn’s disease (CD). 1 The phenotypic features of CD associated with OFG are not well described and there is a paucity of evidence for the use of advanced therapies for OFG.1 The largest case series of anti-TNF therapy used in patients with OFG contains only 14 patients, whilst evidence for other advanced therapies is limited to case reports.2Methods We have retrospectively evaluated the phenotype and clinical response to advanced therapies in individuals with both OFG and CD across two large referral centres. CD phenotype was compared to 2,035 NIHR IBD Bioresource participants recruited from our centres. Statistical analysis was conducted using Fisher’s exact test.Results 116 individuals with OFG and CD were included (58% male, mean follow-up from OFG diagnosis 13.0 years).Prevalent features of OFG included lip swelling (72%), intra-oral ulceration (49%) and cobblestoning of the buccal mucosa (41%).We report high rates of oesophagogastric CD (17% vs 2% in controls, p<0.001) and perianal disease (46% vs 34%, p=0.01). 37% of OFG patients had a stricturing or penetrating phenotype compared to 33% of controls (p=0.42). 43% of OFG patients had undergone resectional surgery.Advanced therapies used for treatment of OFG included adalimumab (n=56), infliximab (n=52), ustekinumab (n=23), risankizumab (n=10), vedolizumab (n=8) and JAK-inhibitors (n=2) (figure 1). Complete or partial response of OFG symptoms was achieved in 75% and 71% of patients receiving adalimumab and infliximab respectively. Remission was achieved at a dose above the standard licenced dose in 15% of those receiving adalimumab and 21% of those receiving infliximab. 69% and 60% of those receiving ustekinumab and risankizumab respectively also achieved complete or partial resolution of OFG symptoms. 50% of those receiving vedolizumab achieved clinical remission.Conclusions We have evaluated phenotype and treatment response in the largest cohort of OFG and CD to date. We have identified that OFG is associated with aggressive CD phenotypes, with high rates of oesophagogastric and perianal disease. OFG responds to anti-TNF therapy in most cases, but may require escalated dosing. >60% of patients receiving ustekinumab and risankizumab also had a clinical response and these agents therefore also represent a viable therapeutic option.References Joshi S, Mawdsley J, Hullah E, Ormond M, Carey B. Management of orofacial granulomatosis. British Journal of Hospital Medicine. 2023;29(3):1–16.Elliott T, Campbell H, Escudier M, et al. Experience with anti-TNF-α therapy for orofacial granulomatosis. J Oral Pathol Med. 2011;40(1):14–19.Abstract P237 Figure 1OFG response to advanced therapies",
  "authors": [
    {
      "affiliations": [
        "Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK",
        "Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK"
      ],
      "name": "Robert Lees"
    },
    {
      "affiliations": [
        "Guy’s and St Thomas’ NHS Foundation Trust, London, UK"
      ],
      "name": "Priyanka Patel"
    },
    {
      "affiliations": [
        "Cruces University Hospital, Bilbao, Spain"
      ],
      "name": "Angelica Molinillo Marin"
    },
    {
      "affiliations": [
        "Guy’s and St Thomas’ NHS Foundation Trust, London, UK"
      ],
      "name": "Cameron Herbert"
    },
    {
      "affiliations": [
        "Guy’s and St Thomas’ NHS Foundation Trust, London, UK"
      ],
      "name": "Esther Hullah"
    },
    {
      "affiliations": [
        "NIHR BioResource, Cambridge, UK"
      ],
      "name": "Laetitia Pele"
    },
    {
      "affiliations": [
        "Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Miles Parkes"
    },
    {
      "affiliations": [
        "Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK",
        "Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK"
      ],
      "name": "Christopher Lamb"
    },
    {
      "affiliations": [
        "Guy’s and St Thomas’ NHS Foundation Trust, London, UK"
      ],
      "name": "Joel Mawdsley"
    },
    {
      "affiliations": [
        "Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK",
        "Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK"
      ],
      "name": "Ally Speight"
    }
  ],
  "title": "P237 Orofacial granulomatosis in patients with crohn’s disease: clinical characteristics and response to advanced therapies in a large multi-centre cohort",
  "uid": "5e7b0dac-c1bc-58f8-848c-981a4ad6dbfb"
}