Systemic Disease and The Mouth Flashcards

1
Q

Why might someone have an immune deficiency?

A

Congenital immune deficiency
Acquired immune deficiency
- Diabetes
- Drug therapy
- Cancer therapy
- HIV

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2
Q

What intra-oral findings might suggest that someone is immune deficient?

A

Candidiasis.
Ulceration on keratinised tissue- suggests jerpes simplex virus reactivation.
Herpes Labialis on the lip.

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3
Q

Why might someone have a haematinic deficiency?

A

Poor oral intake
Malabsorption- GI diseases.
Blood loss- Crohn’s, UC, peptic ulcer disease
Increased demand- childhood growth spurts.

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4
Q

What is systemic Lupus Erythematosis?

A

Complex chronic autoimmune disease with multi system involvement.

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5
Q

What factors are involved in SLE development?

A

Multifactorial involving genetics, and environmental factors.
Complex interaction between environmental factors and genome to produce an epigenetic change that alters expression of specific genes that contribute to the disease developing.

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6
Q

Describe the pathogenesis of SLE.

A

Triggers cause damage to the nucleus or cytoplasm of the cells.
Leads to activation of innate and adaptive immunity, auto reactive B cell activation by T cells, cytokine dysregulation.
Immune complexes deposited into tissues leading to an autoimmune cascade and organ damage.

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7
Q

What are the intra-oral manifestations of SLE?

A

Gingival lesions similar to desquamative gingivitis but red banding does not go as high up into the buccal sulcus.
Oral ulceration.
Hyposalivation
Erosion
Burning mouth syndrome.
Lichen Planus- particularly in the palate.
Cheilitis.

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8
Q

What are the extra-oral manifestations of SLE?

A

Malar rash
Renal abnormalities- protenuria, hematuria.
Ascites
Arthritis
Anaemia
Thrombocytopenia
Lung disease
Raynaud’s.

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9
Q

Describe the histology of SLE.

A

Inflammatory infiltrate (CD4-positive T cells) found around blood vessels in the lamina propria and dermis.

Granuloma formation in the lamina propria.

Thickening of basement membrane.

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10
Q

What are the SLE-specific auto-antibodies?

A

Anti-dsDNA or anti-smith antibody.
Can also get Anti-Ro and Anti-La but these are also found in Sjogren’s.

Anti-ANA

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11
Q

Describe the 2019 ACR/EULAR consensus on SLE diagnosis?

A

The criteria groups 7 clinical and 3 immunological domains together and weights them from 2 to 10.
To be diagnosed with SLE, you need to have a total score of 10 with at least one clinical criterion.

Clinical criterion
- Constitutional- fever
- Serosol
- Renal- Class III or Class IV renal nephritis would give a score of 10, proteinuria greater than 0.5g in 24 hours.
- Neuropsychiatric- delirium, psychosis, seizure
- Musculocutaneous- oral ulcers, non scarring alopecia
- Musculoskeletal- joint involvement
- Haematological- leukopenia, thrombocytopenia, autoimmune haemolysis.

Immunological domains
- Anti-dsDNA or anti-smith antibody
- C3 or C4 complement proteins

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12
Q

Why might someone have hyperpigmentation on their oral mucosa?

A

Melanoma.
Raised ACTH- addison’s disease.
Racial features
Cushing’s
Amalgam tattoo, Chlorhexidine, coffee, red wine, tea.
Melanocytic Naevus

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13
Q

What is orofacial granulomatosis?

A

Clinical presentation of oedema in the oral and facial tissues by blockage of lymphatic drainage by granulomas due to an immune reaction.

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14
Q

What is the difference between angio-oedema and OFG?

A

Angio-oedema is due to an increase in fluid exudate and not due to blockage of lymphatics.
Swelling arises quickly and reduces quickly- within 24 hours.

OFG is due to blockage of lymphatic drainage and not an increase in fluid exudate.
Swelling arises quickly and reduces slowly.

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15
Q

Histologically, what does OFG look like?

A

Fluid accumulation within connective tissue bundles.
Giant cells forming within tissues - blocking the lymphatic system.

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16
Q

What are the signs and symptoms of OFG?

A

Erythema of the peri-oral tissues
Oral and facial swelling- particularly in the lower lip
Angular cheilitis
Full thickness gingivitis- not plaque related.
Ulceration in the labial mucosa- linear fissured ulcer that is not caused by trauma.
Staghorning in the floor of the mouth.

17
Q

If you see the above symptoms, what might you wish to ask the patient?

A

Any abdominal pain?
Altered bowel habits?
Have you grown since you were last here? Have your clothes become too small for you?

18
Q

What investigations might you request if you suspect OFG?

A

Growth monitoring at hospital
Faecal calprotectin assay- if positive then endoscopy indicated.
Diet history- identify potential allergens.

19
Q

In primary care, what might you advise the patient?

A

Exclusion diet trial- benzoic acid, sorbic acid, cinnamon products, chocolate, E210-219.
Do this for at least 3 months and see if the symptoms ease.

Liaise with dietetics if required.

Advise patients about food maestro- good app which shows allergens in specific foods.

20
Q

In a specialist setting, what medical therapy may be indicated for OFG?

A

Topical treatment
- Miconazole cream 2% 20mg tube, apply to area twice a day.
- Tacrolimus ointment to area of peri-oral swelling.

Intralesional steroid injection- Triamcinolone injected binto the area of swelling, usually weekly for 3 weeks.

Systemic treatment to help immune modulation
- Pulsed azithromycin for 3 months, 3 days in every week.
- Prednisolone
- Mycophenolate
- Azothioprine.

21
Q

What dental manifestations might occur in a child with a systemic illness?

A

Congenital conditions/infections
- Ectodermal Dysplasia
- Syphillis

Illness/metabolic disorder
- Cancer treatments
- Diabetes

Pigmentation from substances in the blood
- Bilirubin
- Tetracycline

22
Q

Apart from OFG, what other conditions might cause peri-oral and facial swelling?

A

Tuberculosos
Sarcoidosis
Angio-oedema
Crohn’s disease

23
Q

What type of immune reaction occurs in OFG?

A

Type IV hypersensitivity reaction
- T helper cells are activated as a result of an allergen, they then activate macrophages.
- Macrophages will try get rid of the pathogen but if the pathogen is not removed- the macrophages will join to form a multinucleate giant cell.
- In OFG, these get stuck in lymphatic drainage and cause the swelling to occur.

24
Q

Describe the other hypersensitivity reactions.

A

Type I-
- Angio-oedema.
- granulation of mast cells in response to an allergen.
- Granules and vasoactive components into the tissues, where they act upon local blood vessels to increase vascular permeability.

Type II-
- Blood transfusion reaction, autoimmune haemolytic anaemia.
- Antibody-mediated immune reaction, where antibodies are directed against cellular and ECM antigens.
- Resulting in tissue destruction, functional loss or damage to tissues.

Type III
- Immune-complexes sensitivity.

25
Q

What are the therapeutic options for SLE?

A

First line treatment-
- Hydroxychloroquine 200-400mg daily.

Then IV Methylprednisolone.

Methotrexate or azathioprine.

Monoclonal antibodies- Belimumab, Ritixumab.