Orofacial Granulomatosis Flashcards
What is OFG?
Orofacial Granulomatosis
- clinical presentation of oedema in oral and facial ST by blockage of lymphatic drainage due to an immune reaction
- Angio oedema: oedema of the oral and facial ST due to an increase in fluid exudate from capillaries but with NO lymphatic drainage
- Angio-oedema swelling comes up quickly and settles quickly - usually in 24-48 hours
- OFG swelling come sup quickly and settles only very slowly- weeks/ months
- continuous swelling
- changes in intensity day to day/ week to week
- no increase in fluid exudate
- accumulate within ST due to obstruction of lymphatic drainage
Other granulomatosis disease
- Sarcoidosis - rare
- unless there are sarcoidosis present in other body sites - Tuberculosis - rare
- unless has pulmonary disease - Crohn’s disease- common
- affect any part of GI tract including mouth
Angio- oedema
- fluid present within CT bundles
OFG
- no evidence of tissue swelling
- giant cells forming
- immune reaction around mouth
- crusting of mouth
Type 1 vs 2 vs 3 vs 4
Type 1- allergy and atrophy, ie: angio-oedema
- degranulation of mast cells in response to an allergen
- send vasoactive compounds into tissues where they act upon local BV to increase vascular permeability
Type 2- Antibody mediated hypersensitivity
Type 3- Immune complex mediated hypersensitivity
Type 4- Delayed type hypersensitivity (DTH)
- T cells activated by an allergen and trigger macrophages to become active
Type 4 (cell mediated) hypersensitivity
- trigger of reaction, then T helper cells are then activated
- interact with macrophages to produce activated macrophages
- try to phagocytose the allergen
- if not possible, then macrophages will fused into multinucleated giant cells (MGC)
- will then further phagocytose and remove allergen
About OFG
- can present at any age
- more common in later childhood and adolescent
- often present in low levels; pt unaware
- most are mild and can be controlled with simple measures
Severe and unremitting forms
- extremely disabling for pt
- reduction in QoL
- appearance issues for kids
- lead to depression and withdrawal from social contact when extreme
Facial Changes
Crohn’s screening
- if pt is aware of any abdominal changes/ pain
- growth monitoring
Faecal Calprotectin assay
- unreliable in children
- screening test for endoscopy (need GA)
Initial management of OFG
- Consider whether it is Orofacial or part of Crohns
- GI symptoms
- Faecal Calprotectin assay
- Growth monitoring - Dietary history
- identify if overuse of dietary allergens
- discuss role of a complete exclusion diet trial
Exclusion Diet Trial (primary care/ specialist)
Avoid food containing
- benzoic acid
- sorbic acid
- cinnamon products
- chocolate
- E210-219 not included
- support with dietetics/ other aids
- 100% compliance for 3 months
- test re-introduction of favourite excluded foods
Food Maestro App
Dietary allergen
- Benzoate, Sorbate, Cinnamon, Chocolate
- Benzoates found in tomatoes and tomato products
Medical management (Specialist only)
Topical tx
- Miconazole: Angular Chelitis
- Tacrolimus ointment: areas of lip swelling and facial erythema
Intralesional steroid injection
- Triamcinolone injected into area of swelling
- weekly for 3 weeks
Systemic tx
- help immune modulation
- Pulsed azithromycin for 3 months - 3 days every week
- Prednisolone pulse for short term use
- Azathioprine/ Mycophenolate
