Connective tissue disease Flashcards

1
Q

What is an autoimmune disease?

A
  • Disease that occurs when body’s immune system mistakes own cells for foreign cells, causing the body to attack it’s own healthy cells
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2
Q

What are some examples of autoimmune disease?

A
  • Systemic lupus erythematosis (SLE)
  • Systemic sclerosis (Scleroderma)
  • Sjogrens syndrome
  • Undifferentiated connective tissue disease (UCTD)
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3
Q

What do normal people and people with autoimmune disease have in common?

A
  • They both have associated blood autoantibodies
  • The autoantibodies don’t cause the disease
  • The complement activation causes tissue damage
  • The pattern of the autoantibodies determines the problem
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4
Q

What are vasculitic diseases?

A
  • Form of connective tissue disease
  • Affects the blood vessels
    3 types
  • Large vessel disease
  • Medium vessel disease
  • Small vessel disease
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5
Q

What is an example of large vessel disease?

A
  • Giant cell (temporal) arteritis
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6
Q

What is an example of medium vessel disease?

A
  • Polyarteritis nodosa
  • Kawasaki disease
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7
Q

What is an example of small vessel disease?

A
  • Wegener’s granulomatosis
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8
Q

What is the treatment for all connective tissue disease?

A
  • Analgesic NSAID’s to treat joint/ muscle symptoms
  • Immune modulating treatment to disrupt immune process
  • Systemic steroids used short term to reduce inflammatory process but long term not noticeable
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9
Q

What are some examples of immune modulating treatment?

A
  • Hydroxychloroquine
  • Methotrexate
  • Azathioprine
  • Mycophenolate
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10
Q

What is an example of a systemic steroid used for treatment of connective tissue disorder?

A
  • Prednisolone
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11
Q

What systems are involved in the condition lupus?

A
  • Lupus is involved in every system of body
    Have
  • Sjogren’s syndrome features
  • Raynaud’s phenomenon
  • Rheumatoid arthritis
  • Mixed connective tissue disease
  • Sclerodoma
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12
Q

What are the common antibodies in autoimmune disease?

A
  • Anti-nuclear antibody (ANA)
  • Anti-double-strand DNA (dsDNA)
  • Anti-Ro antibody (Ro)
  • Anti-La antibody (La)
  • Anti - centromere antibody
  • Anti-Sci-70 antibody
  • Anti-Neutrophil cytoplasmic antibody (ANCA)
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13
Q

What is systemic lupus erythematosis?

A
  • Two types (Discoid lupus / Systemic lupus)
  • Tissue change without blood autoantibodies called Discoid lupus which is seen in skin and mouth and looks sim to lichen planus
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14
Q

What are the circulating immune complexes in systemic lupus erythematosis?

A
  • ANA antibodies
  • dsDNA antibodies
  • Ro antibodies
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15
Q

What systems does systemic lupus erythematosis affect?

A
  • Multi system
  • Joints, skin, kidney, muscles and blood
  • CVS, RS and CNS
  • Renal involvement is major cause of death
  • Also have enhanced cardiovascular risk
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16
Q

What is the prevalence of systemic lupus erythematosis?

A
  • 12-64/100,000 population
  • Twins 20-50%
  • Siblings same as non-relatives with 5%
  • Females of child bearing age
  • Photosensitivity common lead to butterfly zygomatic rash seen across face
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17
Q

What are some features of systemic lupus erythematosis?

A
  • Pleural effusions
  • Heart problems
  • Lupus nephritis
  • Arthritis
  • Raynaud’s phenomenon
  • Butterfly rash
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18
Q

What are the dental aspects of SLE?

A
  • If patient has chronic anaemia can lead to oral ulcerations and GA risk
  • If has thrombocytopenia has bleeding tendency
  • If renal disease they have impaired drug metabolism
  • Drugs can trigger photosensitivity
  • If taking steroid and immunosuppressive drugs then possible increased malignancy risk
  • Lichenoid oral reactions presentation but in fact lupus
  • Oral pigmentation from hydroxychloroquine use
19
Q

What is Lupus anticoagulant?

A
  • Marker found in blood of some patients with lupus
  • Indicates a subtype of lupus patient
  • Patients termed antiphospholipid antibody syndrome
  • The marker anticoagulates in test tube but NOT in a patient
20
Q

What are the two forms of antiphospholipid antibody syndrome?

A

Primary form - No other associated disease
Secondary form - Found in some patients with chronic inflammatory conditions e.g. SLE

21
Q

What are the antibodies found in antiphospholipid antibody syndrome (APS) patients?

A
  • Antiphospholipid (aPL)
  • Anticardiolipin (aCL)
22
Q

What is the main characteristic of APS?

A
  • Recurrent thrombosis
  • Put on medications for this (anticoagulants like Warfarin)
  • Do not stop DVT medication as this can lead to pulmonary embolism
23
Q

What is Sjogren’s syndrome?

A
  • Inflammatory disease associated with but not caused by circulating antibodies
  • ANA, Ro and La
  • Associated with dry eyes and dry mouth
  • Major involvement in salivary glands for some which leads to xerostomia and oral disease
24
Q

What are 3 main groups of Sjogren’s syndrome?

A

Sicca syndrome - Only associated with dry eyes or dry mouth

Primary sjogren’s - Not associated with any other disease

Secondary sjogren’s - Associated with another connective tissue disease e.g. RA and SLE

25
Q

What is the diagnostic for Sjogren’s syndrome?

A
  • Not one test that proves you have it
  • Some more imp than others
    Dry eyes/ mouth
    Autoantibody findings (ANA and Anti-ro)
    Imaging findings
    Histopathology findings (from labial gland biopsy)
26
Q

What are the dental implications of Sjogren’s syndrome?

A
  • Oral infection
  • Caries risk
  • Functional loss
  • Denture retention
  • Sialosis
  • Salivary lymphoma (unilateral gland changes usually after years of diagnosis)
27
Q

Why does systemic sclerosis occur?

A
  • Due to excessive collagen deposition
  • Connective tissue fibrosis
  • Loss of elastic tissue internal and external
  • Mainly affects women
28
Q

What are the two forms of systemic sclerosis?

A

Local - anticentromere antibodies
Generalised - anti sci-70 antibodies

29
Q

What are some features of systemic sclerosis?

A
  • Telangiectasia
  • Raynauds
  • Nailfold capillaroscopy
  • Sclerodactyly
30
Q

What is the onset of systemic sclerosis?

A
  • Gradual onset
  • Starts with Raynaud’s phenomenon
  • Renal failure
  • Malabsorption with GI involvement
  • No treatment
31
Q

What are the dental aspects of systemic sclerosis?

A
  • Loss of elasticity which has involvement of cheek and perioral tissues
  • Leads to limited mouth opening
  • Progressively poor oral access
  • Limited tongue movement
  • Need to plan treatment 10years ahead
  • May be compounded by Sjogrens with dry mouth
32
Q

What are some other dental aspects of systemic sclerosis?

A

Dysphagia and relfux oesophagitis
- Swallowing difficulties
- Dental erosion

Cardiac and renal vasculitic disease
- Watch drug metabolism

Widening of PDL space
- No dental mobility

33
Q

What are some other dental aspects of systemic sclerosis?

A

Dysphagia and reflux oesophagitis
- Swallowing difficulties
- Dental erosion

Cardiac and renal vasculitis disease
- Watch drug metabolism

Widening of PDL space
- No dental mobility

34
Q

What is Vasculitis?

A
  • Inflammation of blood vessels
  • Infarction of tissue (may present as ulcers due to tissue necrosis/ oral inflammatory masses)
  • Vessel wall thickens with inflammation
  • Narrowing of lumen reduces blood flow
35
Q

What is Giant cell arteritis?

A
  • AKA temporal arteritis as commonly temporal artery is involved
  • Large vessel disease vasculitis disease
36
Q

Why is it important to diagnose giant cell arteritis early on and what are the warning signs?

A
  • May present with headache/ facial pain
  • Involves carotid branches
  • Occlusion of central retinal artery can cause blindness
  • It is a facial pain emergency and should be started on immune suppressant like prednisolone as soon as poss
37
Q

What is polymyalgia rheumatica?

A
  • Disease of the elderly (60-70years)
  • Large vessel vasculitis
38
Q

What are the signs and symptoms of polymyalgia rheumatica?

A
  • Pain and morning stiffness in muscles
  • Malaise
  • Weight loss
  • Profound fatigue
  • Responds well to steroids
39
Q

What is Kawasaki disease?

A
  • Mainly a disease of children
  • AKA mucocutaneous lymph node syndrome
  • Medium vessel vasculitis
40
Q

What are the clinical signs of Kawasaki disease?

A
  • Fever and lymphadenopathy
  • Crusting/cracked tongue
  • Strawberry tongue and erythematous mucosa
  • Peeling rash on hands and feet
  • Can develop coronary artery aneurysm and will require antibiotic prophylaxis but this is determined by cardiologist
41
Q

What is Wegener’s granulomatosis?

A
  • Inflammatory condition
  • Small vessel vasculitis
  • Can lead to destruction of hard and soft tissue of face and oral cavity
  • Spongey red tissue inside mouth (looks like vascular red rash over gingiva)
  • Associates with ANCA
  • Refer for assessment
42
Q

What is fibromyalgia?

A
  • Non specific collection of musculoskeletal symptoms
  • Joint pain
  • Muscle pain
  • Functional disorder where patient perceives pain where no infection is acc present
43
Q

What are the dental aspects of vasculitis diseases?

A
  • Steroid precautions may be needed
  • Giant cell arteritis/ Wegener’s granulomatosis and Kawasaki disease may present to us first