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
2
Q
What are some examples of autoimmune disease?
A
- Systemic lupus erythematosis (SLE)
- Systemic sclerosis (Scleroderma)
- Sjogrens syndrome
- Undifferentiated connective tissue disease (UCTD)
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
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
5
Q
What is an example of large vessel disease?
A
- Giant cell (temporal) arteritis
6
Q
What is an example of medium vessel disease?
A
- Polyarteritis nodosa
- Kawasaki disease
7
Q
What is an example of small vessel disease?
A
- Wegener’s granulomatosis
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
9
Q
What are some examples of immune modulating treatment?
A
- Hydroxychloroquine
- Methotrexate
- Azathioprine
- Mycophenolate
10
Q
What is an example of a systemic steroid used for treatment of connective tissue disorder?
A
- Prednisolone
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
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)
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
14
Q
What are the circulating immune complexes in systemic lupus erythematosis?
A
- ANA antibodies
- dsDNA antibodies
- Ro antibodies
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
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
17
Q
What are some features of systemic lupus erythematosis?
A
- Pleural effusions
- Heart problems
- Lupus nephritis
- Arthritis
- Raynaud’s phenomenon
- Butterfly rash
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
What is the diagnostic for Sjogren's syndrome?
- 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
What are the dental implications of Sjogren's syndrome?
- Oral infection
- Caries risk
- Functional loss
- Denture retention
- Sialosis
- Salivary lymphoma (unilateral gland changes usually after years of diagnosis)
27
Why does systemic sclerosis occur?
- Due to excessive collagen deposition
- Connective tissue fibrosis
- Loss of elastic tissue internal and external
- Mainly affects women
28
What are the two forms of systemic sclerosis?
Local - anticentromere antibodies
Generalised - anti sci-70 antibodies
29
What are some features of systemic sclerosis?
- Telangiectasia
- Raynauds
- Nailfold capillaroscopy
- Sclerodactyly
30
What is the onset of systemic sclerosis?
- Gradual onset
- Starts with Raynaud's phenomenon
- Renal failure
- Malabsorption with GI involvement
- No treatment
31
What are the dental aspects of systemic sclerosis?
- 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
What are some other dental aspects of systemic sclerosis?
Dysphagia and relfux oesophagitis
- Swallowing difficulties
- Dental erosion
Cardiac and renal vasculitic disease
- Watch drug metabolism
Widening of PDL space
- No dental mobility
33
What are some other dental aspects of systemic sclerosis?
Dysphagia and reflux oesophagitis
- Swallowing difficulties
- Dental erosion
Cardiac and renal vasculitis disease
- Watch drug metabolism
Widening of PDL space
- No dental mobility
34
What is Vasculitis?
- 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
What is Giant cell arteritis?
- AKA temporal arteritis as commonly temporal artery is involved
- Large vessel disease vasculitis disease
36
Why is it important to diagnose giant cell arteritis early on and what are the warning signs?
- 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
What is polymyalgia rheumatica?
- Disease of the elderly (60-70years)
- Large vessel vasculitis
38
What are the signs and symptoms of polymyalgia rheumatica?
- Pain and morning stiffness in muscles
- Malaise
- Weight loss
- Profound fatigue
- Responds well to steroids
39
What is Kawasaki disease?
- Mainly a disease of children
- AKA mucocutaneous lymph node syndrome
- Medium vessel vasculitis
40
What are the clinical signs of Kawasaki disease?
- 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
What is Wegener's granulomatosis?
- 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
What is fibromyalgia?
- Non specific collection of musculoskeletal symptoms
- Joint pain
- Muscle pain
- Functional disorder where patient perceives pain where no infection is acc present
43
What are the dental aspects of vasculitis diseases?
- Steroid precautions may be needed
- Giant cell arteritis/ Wegener's granulomatosis and Kawasaki disease may present to us first
