TY - JOUR
T1 - Orofacial Granulomatosis Associated with Crohn's Disease
T2 - a Multicentre Case Series
AU - Phillips, Frank
AU - Verstockt, Bram
AU - Sladek, Malgorzata
AU - de Boer, Nanne
AU - Katsanos, Konstantinos
AU - Karmiris, Konstantinos
AU - Albshesh, Ahmad
AU - Erikson, Carl
AU - Bergemalm, Daniel
AU - Molnar, Tamas
AU - Ellul, Pierre
N1 - Publisher Copyright: © 2021 The Author(s).
PY - 2022/3/1
Y1 - 2022/3/1
N2 - 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.
AB - 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.
KW - Crohn’s disease
KW - Orofacial granulomatosis
KW - inflammatory bowel disease
KW - oral Crohn’s disease
KW - ulcerative colitis
UR - http://www.scopus.com/inward/record.url?scp=85126490988&partnerID=8YFLogxK
U2 - https://doi.org/10.1093/ecco-jcc/jjab158
DO - https://doi.org/10.1093/ecco-jcc/jjab158
M3 - Article
C2 - 34498037
SN - 1873-9946
VL - 16
SP - 430
EP - 435
JO - Journal of Crohn's & Colitis
JF - Journal of Crohn's & Colitis
IS - 3
ER -