Orofacial Granulomatosis Associated with Crohn’s Disease: a Multicentre Case Series

Phillips, Frank; Verstockt, Bram; Sładek, Małgorzata; Boer, Nanne de; Κατσάνος, Κωνσταντίνος; Karmiris, Konstantinos; Albshesh, Ahmad; Erikson, Carl A.; Bergemalm, Daniel; Molnár, Tamás; Ellul, Pierre; Investigators, Ecco Confer

doi:10.1093/ecco-jcc/jjab158

Cited by 7 publications

(4 citation statements)

References 24 publications

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“…Thus, perianal disease was 2.5-times more prevalent in patients with CD-OFG than in the CD-Ref group [16/25 (64%) versus 13/50 (26%)], with the latter percentage being similar to those reported previously in follow-up studies of pediatric CD cohorts [29,30]. A high prevalence of perianal disease in predominantly adult patients with CD and concomitant OFG was also reported in a recently published multicenter study [8]. Notably, the high prevalence of perianal disease in the CD-OFG group was reflected in the numerous perianal surgical procedures in this diagnostic group, in that about half of the patients with CD-OFG underwent such procedures, as compared to only one-tenth of the patients in the CD-Ref group, with the latter value being in line with that reported in a recent populationbased Swedish study [31].…”

Section: Discussionsupporting

confidence: 87%

“…OFG may appear as a separate disorder or in conjunction with a systemic disease such as sarcoidosis [ 5 ] or CD [ 6 ]. The coexistence of OFG with CD is most commonly seen in children and young adults [ 7 ], although it occurs in adults of all ages [ 6 , 8 ]. OFG may precede, occur concomitantly with or develop after the onset of the gastrointestinal manifestations of CD [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

“…Our research group has previously reported that at the time of CD diagnosis, patients with childhood-onset CD with concomitant OFG (CD-OFG) have a higher prevalence of perianal disease (48% vs 18%) [ 19 ], and display intestinal granulomas more than twice as often as patients with CD but without OFG (81% vs 38%) [ 19 ]. High prevalence rates of perianal disease have also been reported for patients with CD-OFG in the UK [ 6 ], Ireland [ 20 ] and France [ 21 ], as well as in a recently published multicenter study [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Frequent Occurrence of Perianal Disease and Granuloma Formation in Patients with Crohn’s Disease and Coexistent Orofacial Granulomatosis

et al. 2023

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Background Orofacial granulomatosis (OFG) is an inflammatory disorder of the perioral region and oral cavity. Crohn's disease (CD) in conjunction with OFG (CD-OFG), has been suggested to constitute a phenotype of CD with distinct features at diagnosis. Aims The aim of this project was to investigate whether the distinct phenotypic features of CD-OFG persist in the years following the initial diagnosis of CD. Methods Clinical data were extracted from medical records covering the first 5 years post-diagnosis for a cohort of patients with CD-OFG, and were compared to those of references with CD without OFG. ResultsThe clinical characteristics of our cohort of patients with CD-OFG (N = 25) were evaluated in comparison to references with CD without OFG (ratio 1:2). Five years post-diagnosis, more patients with CD-OFG had a phenotype with perianal disease (cumulative incidence: 16/25, 64% vs 13/50, 26%, P = 0.002) and intestinal granulomas (cumulative incidence: 22/25, 88% vs 24/50, 48%, P = 0.0009) than patients in the CD reference group. The patients with CD-OFG were also more likely to have undergone perianal surgery (12/25, 48% vs 4/50, 8%, P = 0.0002). At the end of the observation period, more of the patients with CD-OFG were receiving combination therapy, i.e., immunomodulators and tumor necrosis factor antagonists, than those in the CD reference group (9/25, 36% vs 5/50, 10%, P = 0.01). ConclusionThe results support the notion that CD in conjunction with OFG represents a specific phenotype of CD that is characterized by frequent perianal disease, pronounced intestinal granuloma formation and a need for extensive therapy.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Frequent Occurrence of Perianal Disease and Granuloma Formation in Patients with Crohn’s Disease and Coexistent Orofacial Granulomatosis

et al. 2023

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“…TNF‐α also may stimulate cutaneous recruitment of inflammatory cells via enhanced vasculature expression of adhesion molecules, including the metalloproteinases MMP‐9 and MMP‐10 21,24,25. The use of anti‐TNF‐α biologics as first‐line therapy for IBD‐associated PG has been recommended, as PG severity may be independent of IBD clinical activity 15,42–46. Clinical reports also have demonstrated the efficacy of infliximab and adalimumab therapy of PG in other immune‐mediated disorders,19,30,33 The nine reported pediatric and adolescent patients who received an anti‐TNF‐α biologic agent for IBD‐associated PG are listed in Table 3.…”

Section: Treatment Of Pyoderma Gangrenosummentioning

confidence: 99%

Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease

Vaidy¹,

Winderman²,

Rabinowitz³

et al. 2020

JPGN Reports

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Pyoderma gangrenosum (PG) is a rare, necrotizing dermatologic condition associated with neoplastic and immune dysregulatory states, including adult and pediatric inflammatory bowel disease (IBD). Over the last decade, the elucidation of inflammatory mediators in PG has led to a plethora of localized and systemic corticosteroid sparing therapies including antibiotics, antiinflammatory, and immunomodulatory agents. Herein, we describe the case of a 17-year-old female with ulcerative colitis in clinical remission, who presented with a long-standing, large, deep, and painful lower extremity PG lesion. Following failed attempts both at local and at systemic therapies, her PG was successfully treated with the tumor necrosis factor-alpha (TNF-α) monoclonal antibody adalimumab, and the lesion remains in remission after four years of subcutaneous anti-TNF therapy. This case serves as the basis for our presenting a review of the pathogenesis, diagnostic criteria, differential diagnosis, therapies and treatment outcomes for pediatric IBD-associated PG. Our experience adds to earlier reports suggesting anti-TNF-α biologic therapy is most likely to achieve long-term resolution of IBD-associated PG in children and adolescents with severe lesions or who failed other treatments.

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