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

2021 ◽  
Author(s):  
Frank Phillips ◽  
Bram Verstockt ◽  
Malgorzata Sladek ◽  
Nanne de Boer ◽  
Konstantinos Katsanos ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Case Reports ◽  
Case Series ◽  
Symptom Burden ◽  
Management Options ◽  
Orofacial Granulomatosis ◽  
Tract Involvement ◽  
Collection Form ◽  
Upper Gastrointestinal

Abstract Background Orofacial granulomatosis [OFG] is a rare syndrome that may be associated with Crohn’s disease [CD]. We aimed to characterise this relationship and the management options in the biologic era. Methods This multicentre case series was 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. Clinical data were recorded in a standardised collection form. Results This report includes 28 patients with OFG associated with CD: 14 males (mean age of 32 years, ±12.4 standard deviation [SD]) and 14 females [40.3 years, ±21.0 SD]. Non-oral upper gastrointestinal tract involvement was seen in six cases and perianal disease in 11. The diagnosis of OFG was made before CD diagnosis in two patients, concurrently in eight, and after CD diagnosis in 18. The distribution of OFG involved the lips in 16 cases and buccal mucosa in 18. Pain was present in 25 cases, with impaired swallowing or speaking in six. Remission was achieved in 23 patients, notably with the use of anti-tumour necrosis factors [TNFs] in nine patients, vedolizumab in one, ustekinumab in one, and thalidomide in two. A further five cases were resistant to therapies including anti-TNFs. Conclusions OFG associated with CD may occur before, concurrently with, or after the diagnosis of CD. Perianal and upper gastrointestinal [UGI] disease are common associations and there is a significant symptom burden in many. Remission can be obtained with a variety of immunosuppressive treatments, including several biologics approved for CD.

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

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S209-S210
Author(s):  
F Phillips ◽  
B Verstockt ◽  
M Sladek ◽  
N de Boer ◽  
K Katsanos ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Psychological Distress ◽  
Crohn's Disease ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Perianal Disease ◽  
Exclusive Enteral Nutrition ◽  
Orofacial Granulomatosis ◽  
Pyostomatitis Vegetans

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 common associations, and there is a significant symptom burden due to pain and impaired swallowing, leading to psychological distress in many. Remission can be obtained with a variety of treatments, including topical and intralesional agents, biologic agents, and thalidomide.

Tu1225 Upper Gastrointestinal Tract Involvement in Crohn's Disease Requiring Anti-TNF Therapy: Clinical Characteristics

2012 ◽  
Vol 142 (5) ◽  
pp. S-779
Author(s):  
William J. Tremaine ◽  
William A. Faubion ◽  
Darrell S. Pardi ◽  
Lawrence J. Timmons ◽  
Sunanda V. Kane ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Gastrointestinal Tract ◽  
Crohn's Disease ◽  
Clinical Characteristics ◽  
Upper Gastrointestinal Tract ◽  
Tract Involvement ◽  
Upper Gastrointestinal

scholarly journals Diagnosis and Outcome of Oesophageal Crohn’s Disease

2019 ◽  
Vol 14 (5) ◽  
pp. 624-629
Author(s):  
Rita Vale Rodrigues ◽  
Margaret Sladek ◽  
Konstantinos Katsanos ◽  
C Janneke van der Woude ◽  
Juan Wei ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Case Reports ◽  
Case Series ◽  
Disease Diagnosis ◽  
Mucosal Healing ◽  
Case Report Form ◽  
Endoscopic Remission ◽  
Mean Time

Abstract Background and Aims Crohn’s disease [CD] can involve any part of the gastrointestinal tract. We aimed to characterize the clinical, endoscopic and histological features and treatment outcomes of CD patients with oesophageal involvement. Methods We collected cases through a retrospective multicentre European Crohn’s and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. Clinical data were recorded in a standardized case report form. Results A total of 40 patients were reported (22 males, mean [±SD, range] age at oesophageal CD diagnosis: 25 [±13.3, 10–71] years and mean time of follow-up: 67 [±68.1, 3–240] months). Oesophageal involvement was established at CD diagnosis in 26 patients [65%] and during follow-up in 14. CD was exclusively located in the oesophagus in two patients. Thirteen patients [32.2%] were asymptomatic at oesophageal disease diagnosis. Oesophageal strictures were present in five patients and fistulizing oesophageal disease in one. Eight patients exhibited granulomas on biopsies. Proton-pump inhibitors [PPIs] were administered in 37 patients [92.5%]. Three patients underwent endoscopic dilatation for symptomatic strictures but none underwent oesophageal-related surgery. Diagnosis in pre-established CD resulted in treatment modifications in 9/14 patients. Clinical remission of oesophageal disease was seen in 33/40 patients [82.5%] after a mean time of 7 [±5.6, 1–18] months. Follow-up endoscopy was performed in 29/40 patients and 26/29 [89.7%] achieved mucosal healing. Conclusion In this case series the endoscopic and histological characteristics of isolated oesophageal CD were similar to those reported in other sites of involvement. Treatment was primarily conservative, with PPIs administered in the majority of patients and modifications in pre-existing inflammatory bowel disease-related therapy occurring in two-thirds of them. Clinical and endoscopic remission was achieved in more than 80% of the patients.

scholarly journals Upper Gastrointestinal Tract Involvement in Crohn’s Disease: Frequency, Risk Factors, and Disease Course

2018 ◽  
Vol 12 (12) ◽  
pp. 1399-1409 ◽  
Author(s):  
Thomas Greuter ◽  
Alberto Piller ◽  
Nicolas Fournier ◽  
Ekaterina Safroneeva ◽  
Alex Straumann ◽  
...  
Keyword(s):  
Risk Factors ◽  
Crohn’S Disease ◽  
Gastrointestinal Tract ◽  
Crohn's Disease ◽  
Upper Gastrointestinal Tract ◽  
Disease Course ◽  
Tract Involvement ◽  
Upper Gastrointestinal

scholarly journals Granuloma-Positive Crohn’s Disease

2007 ◽  
Vol 21 (9) ◽  
pp. 583-587 ◽  
Author(s):  
Hugh J Freeman
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Population Database ◽  
Classification Schemes ◽  
Epithelioid Granuloma ◽  
Disease Site ◽  
Tract Involvement ◽  
Biological Event ◽  
Present Evaluation ◽  
Upper Gastrointestinal

BACKGROUND: The epithelioid granuloma is a characteristic histological feature of Crohn’s disease. In some pathological classification schemes, the criteria for a definite, probable or possible diagnosis have been listed, with the epithelioid granuloma indicating definite Crohn’s disease.METHODS: In the present evaluation, 247 prospectively evaluated Crohn’s disease patients (24.3%), from a consecutively accumulated population database of 1015 patients, were found to have an epithelioid granuloma. The recently devised Montreal classification for Crohn’s disease was then applied to this granuloma-positive cohort of Crohn’s disease patients to define age at diagnosis for men and women, disease site and disease behaviour.RESULTS: The investigation showed that patients with Crohn’s disease and granulomas were most often diagnosed early in the course of their disease, particularly women. Their disease was often extensive, with ileocolonic and upper gastrointestinal tract involvement. Finally, disease behaviour was most often complex, especially with penetrating disease complications.CONCLUSION: Using homogeneous (ie, ‘reagent-grade’) patient cohorts defined by a recently devised classification method for Crohn’s disease, the study demonstrated that an epithelioid granuloma may represent a histopathological marker for an early biological event in the etiopathogenesis of Crohn’s disease, and this may have predictive significance with respect to the location and clinical behaviour of Crohn’s disease.

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