Rheumatology - Connective Tissue Diseases Flashcards
(95 cards)
1
Q
Connective tissue diseases are broken down into two groups. What are these?
A
- Autoimmune diseases
- Vascular diseases
2
Q
What are the targets of connective tissue diseases?
A
- The targets in CTD are often the tissues themselves whereas in the vasculitic vessel disease it is primarily the blood vessels which are the target of the inflammation
3
Q
Give examples of multisystem vasculitic inflammatory diseases?
A
- Systemic lupus erythematosis
- Systemic sclerosis (Scleroderma)
- Sjogren’s syndrome
- Undifferentiated connective tissue disease
4
Q
Each of the multisystem vasculitis inflammatory diseases have associated blood antibodies. Explain this? (3)
A
- These do not cause the disease - they are found in ‘normal’ people
- Pattern varies from disease to disease
- There are some healthy people who never go on to develop the diseases however the diseases will happen largely in people who have specific autoantibodies and therefore when a patient presents with evidence of one of these autoimmune diseases the pattern of antibodies present in the blood will suggest what direction the disease is going to follow and therefore which category (such as lupus etc) is actually going to be the problem
5
Q
What does complete activation of a multisystem vasculitic inflammatory disease cause?
A
- This causes tissue damage
6
Q
CTD’s in the vasculitic disease category (where the problem is in the blood vessel) are categorised by the size of vessel involved. What are the 3 classifications?
A
- Large vessel disease
- Medium vessel disease
- Small vessel disease
7
Q
Give an example of a ‘large vessel’ vasculitis disease?
A
- Giant cell (temporal) arteritis
8
Q
Give examples of a ‘medium vessel’ vasculitic disease? (2)
A
- Polyarteritis nodosa
- Kawasaki disease
9
Q
Give an example of a ‘small vessel’ vasculitic disease?
A
- Wegener’s Granulomatosis
10
Q
For each of the CTD’s the management is broadly the same. There is no cure for any of the conditions because the cause is not clear. Give examples of ways in which we can manage these conditions? (5)
A
- Dependent on disease activity - immune suppression
- Analgesic NSAID’s
- Immune modulating treatment
- Biologic medication
- Systemic steroids
11
Q
One possible way of managing CTD’s is by using analgesic NSAID’s. What do these do to manage the condition?
A
- These remove joint and muscle symptoms of pain
12
Q
One possible way of managing CTD’s is by using immune modulating treatment. Give examples of these? (4)
A
- Hydroxychloroquine
- Methotrexate
(top 2 are fist line of treatment) - Azathioprine
- Mycophenolate
13
Q
If immune modulating treatment isn’t enough, One possible way of managing CTD’s is by using biologic medication. What do these do to manage the condition and give examples of these? (3)
A
- These are used to disrupt the immune processes
- Examples are cytokine inhibitors and lymphocyte depleting drugs
- (synthetically produced antibodies against certain inflammatory/lymphocyte markers)
14
Q
One possible way of managing CTD’s is by using systemic steroids. What do these do to manage the condition and give an example?
A
- Example: Prednisolone
- There can be used at any stage of the disease in the short term to reduce the inflammatory process and acquire control of the disease process
- But in the longer term the side effects of steroids preclude their regular use
15
Q
One key thing to understand about the immune connective tissue diseases is that they are a spectrum disorder. Explain this?
A
- One way to consider them is lupus as being the jackpot - you have got every possible system involved
- And when you move to the individual diseases at the side specific features are more prominent but within that disease other features of the other diseases may also be present
- We have already looked at RA and we know that it largely affects joints and the peripheral tissues of the body but we also know that patient who have RA can have features of Sjogren’s syndrome
- And in this case the RA is picking from the general connective tissue pool - marked lupus
- But the features that are overlapping for each individual patient may well be different
- A patient with lupus can have features of any of these conditions in addition to inflammatory organ disease more commonly associated with lupus
- Be aware that these are not individual conditions but interlinked and overlapping problems and each patient’s presentation may well be different
16
Q
Give examples of antibodies that can be found in autoimmune diseases? (7)
A
- Anti-nuclear antibody (ANA)
- Anti-double strand DNA (dsDNA)
- Anti-Ro antibody (Ro)
- Anti-La antibody (La)
- Anti-centromere antibody
- Anti-Scl-70 antibody
- Anti-neutrophil cytoplasmic antibody (ANCA)
17
Q
Give examples of the 4 antibodies commonly found in most of the connective tissue diseases?
A
- Anti-nuclear antibody
- Anti-double-strand DNA
- Anti-Ro antibody
- Anti-La antibody
18
Q
Give examples of the 3 antibodies that are commonly found in some overlapped symptoms?
A
- Anti-nuclear antibody
- Anti-double-strand DNA
- Anti-Ro antibody
19
Q
Give examples of the 2 antibodies that are more commonly found in Scleroderma?
A
- Anti-centromere antibody
- Anti-Scl-70 antibody
20
Q
Systemic lupus erythematosis has 2 forms. What are these?
A
- One is called discoid lupus which is largely the same tissue involvement but is much more localised to certain areas of the skin and mouth
- Where there are the organ changes plus the autoantibodies we call this systemic lupus erythematosis
21
Q
What is Discoid lupus?
A
- Tissue changes without blood autoantibodies
- Seen in skin and the mouth - looks similar to lichen planus
22
Q
What are the circulating immune complexes in systemic lupus erythematosis?
A
- ANA
- dsDNA
- Ro antibodies
- (these do not have to be present but they are more likely to be present)
23
Q
Systemic lupus erythematosis is a multi-system disease. What systems can it involve? (4)
A
- Joints, skin, kidneys, muscles, blood
- CVS, RS, CNS
- Renal involvement previously major cause of death
- Enhanced cardiovascular risk
24
Q
IS systemic lupus erythematosis a genetic or an environmental condition?
A
- IT is partly a genetic condition but there is also an environmental trigger
- It is though that there is an infective process which triggers the immune reaction
25
How many people out of the population are affected by systemic lupus erythematosis?
12-64/100,000 population
26
Who in the population is systemic lupus erythematosis seen in?
- Females of childbearing age
27
What is a common feature of systemic lupus erythematosis?
- Photosensitivity common
- Butterfly zygomatic rash
- Typical external features
- Patients who have lupus will generally have a genetic skin photosensitivity and as a general rule tend to avoid sunshine
28
What are the possible dental aspects of SLE? (8)
Chronic anaemia:
- Oral ulceration
- GA risk
Bleeding tendency:
- Thrombocytopenia
Renal disease:
- Impaired drug metabolism
Drug reactions:
- Drugs which are normally innocuous to many dental patients can trigger photosensitivity
Steroid & immunosuppressive therapy
- Possible increased malignancy risk
- Lichenoid oral reactions (looks like lichen planus but is lupus disease in the mucosa)
- Oral pigmentation from hydroxychloroquine use
29
What is lupus anticoagulant?
- This is not a true anticoagulant because it does not make the patients blood less likely to clot but when the blood is placed in a test tube rather than in a patient the blood shows a tendency towards anticoagulation
- Indicates a 'subtype' of lupus patient
30
What are the subtype of lupus patient with lupus anticoagulant termed?
- They are termed 'antiphospholipid antibody syndrome'
31
There are 2 forms of antiphospholipid antibody syndrome - primary and secondary. What is the primary form?
- No other associated disease
32
There are 2 forms of antiphospholipid antibody syndrome - primary and secondary. What is the secondary form?
- Found in some patients with chronic inflammatory conditions e.g. SLE
33
What are the antibodies found in someone with lupus anticoagulant? (2)
- Antiphospholipid (aPL)
| - Anticardiolipin (aCL)
34
What is antiphospholipid antibody syndrome (APS) characterised by?
- One of the key things about AAS is although they have this badly named lupus anticoagulant, they are actually hypercoagulable - they are thrombophilic
- Therefore patients who have this syndrome have a tendency to form DVT and pulmonary emboli without any other risk factors
- These patients are therefore commonly anticoagulated and on warfarin and manage well with this, however if the anticoagulant is stopped for any reason the thrombosis risk will quickly come back and the patient may well have a fatal pulmonary embolus
- Therefore in any patient taking warfarin for APS the dentist must never stop the warfarin without the advice of a physician
35
What is Sjogren's syndrome?
- Inflammatory disease ASSOCIATED with (but not caused by) circulating autoantibodies
- ANA, Ro and La
36
What is Sjogren's syndrome mainly associated with? (2)
- Dry eyes and dry mouth
37
Sjogren's syndrome can be a multi-system disease in some. What else may this involve? (2)
- Major involvement in salivary glands
| - Leads to xerostomia and oral disease
38
Sjogren's syndrome is often broken down into 3 main groups. What are these?
- Sicca syndrome
- Primary Sjogren's
- Secondary Sjogren's
39
What is Sicca syndrome?
- Dry eyes and dry mouth
40
What is primary Sjogren's?
- It is not associated with any other disease
41
What is secondary Sjogren's?
- IT is associated with another connective tissue disease - RA, SLE etc
42
What are the diagnostic criteria for Sjogren's syndrome? (7)
- No one test that 'proves' Sjogren's syndrome
Some signs/symptoms are more important than others:
- Dry eyes/mouth (subjective or objective)
- Autoantibody findings
- Imaging findings
- Histopathology findings
- Positive criteria for diagnosis often a clinical judgement
43
Do we have tests that can prove a person has Sjogren's syndrome?
- No there is no one test that 'proves' a person has Sjogren's syndrome
44
Can a person with Sjogren's syndrome present without dry eyes and mouth?
- If patient does not have dry eyes and dry mouth it makes the diagnosis of Sjogren's seem unlikely but it is possible the patient does in deed have the disease because changes in the lacrimal and salivary glands take 30 years to have their full effect and the patient has the disease process during this entire time
- Therefore if the glands were to be examined histologically before the salivary function had completely exhausted itself there would be evidence of inflammatory change and therefore S patients can indeed have S without dry eyes or dry mouth
45
Which autoantibodies will a person likely have if they have Sjogren's syndrome? (2)
- Antinuclear and anti Ro antibody
46
What is often the most guaranteed method for identifying Sjogren's syndrome?
- Histopathology findings
47
What are possible oral and dental implications of Sjogren's disease? (6)
- Oral infection
- Caries risk
- Functional loss
- Denture retention
- Sialosis (swelling of the salivary glands)
- Salivary lymphoma (unilateral gland size change, usually after years)
48
What is Sialosis?
- Swelling of the salivary glands
49
A salivary lymphoma may occur due to Sjogren's disease. Why does this occur?
- It occurs as a result of chronic activation of the lymphoid tissue
50
What is systemic sclerosis? (4)
- This occurs when the elastic tissue of the body is replaced with connective fibrous tissue and therefore the patient looses the ability to stretch and bend tissues such as the skin as joints and muscles move
- Excessive collagen deposition
- Connective tissue fibrosis
- Loss of elastic tissue
51
What are the 2 different forms of systemic sclerosis?
- Local or generalised form
52
What antibodies are found in the local form of systemic sclerosis?
- Anticentromere antibodies
53
What antibodies are found in the generalised form of systemic sclerosis?
- Anti-Scl-70 antibodies
54
What is it important to remember with systemic sclerosis in terms of which tissues are affected?
- Important to remember that although the loss of this elastic tissue is perhaps most obvious externally it is also lost internally so that organs such as the oesophagus will not stretch to allow the food bolus to pass and the gut will not allow easy peristalsis of the bolus therefore these patients have many hidden problems although their basic disease process does not look so troublesome on the outside
55
What are some common features that are seen in a person with systemic sclerosis? (4)
- Sclerodactyly (contraction of the fingers as the skin will no longer stretch around the joints)
- Raynaud's
- Telangiectasia
- Nailfold capillaroscopy
56
What is Sclerodactyly?
- A contraction of the fingers as the skin will no longer stretch around the joints
57
What is Telangiectasia?
- A condition characterized by dilatation of the capillaries causing them to appear as small red or purple clusters, often spidery in appearance, on the skin or the surface of an organ.
58
What is Nailfold Capillaroscopy?
- Nailfold capillaroscopy is a highly sensitive, inexpensive, simple, safe, and non-invasive imaging technique used in the morphological analysis of nourishing capillaries in the nailfold area . The early detection of microvascular changes that can occur in some inflammatory connective tissue diseases is the main advantage of capillaroscopy, which has attracted the attention of many rheumatologists.
59
Is it predominantly men or women who are affected by systemic sclerosis?
- Women
60
Systemic sclerosis has a gradual onset, what can it result in? (3)
- Raynoud's phenomenon
- Renal failure de to vasculitic damage
- Malabsorption (GI involvement)
61
What is a key feature of systemic sclerosis?
Reynaud's
62
Systemic sclerosis has a slow progression. Is there any treatment available to prevent SS?
- No
63
Explain what happens to the blood vessel patency in the fingers in a patient with systemic sclerosis?
- This is what happens to the BV patency in the fingers during an attack
- The BV's narrow considerably resulting in ischaemia of the fingers which become white
- They go blue as the vessels dilate to keep blood in the tissues
- And finally go red with pain as vascular reperfusion occurs
64
What are the dental aspects/considerations of systemic sclerosis? (6)
There is involvement of perioral tissues
- Limited mouth opening
- Progressively poor oral access
- Limited tongue movement
- Plan treatment 10 years ahead!!!!
- May be compounded by Sjogren's (this will make a caries problem on top of the existing access trouble)
65
What are other dental aspects of systemic sclerosis? (3)
- Dysphagia & reflux oesophagitis (causes difficulty swallowing and dental erosion)
- Cardiac and renal Vasculitic disease (watch drug metabolism)
- Widening of periodontal ligament space (so no dental mobility due to the changes in connective tissue constituents)
66
What happens in vasculitic CTD's?
- In these the arteries of different sizes have inflammation within their walls
- IT is the providing main arterial supply that has the problem and the inflammation in the BV wall causes a significant narrowing of the blood to the tissues leading to ischaemia and changes related to degeneration
67
Vasculitic CTD's can be divided into 3 sub-groups, what are these?
- Large vessel disease
- Medium vessel disease
- Small vessel disease
68
Give examples of large vessel vasculitic diseases? (2)
- Gant cell (temporal) arteritis
| - Polymyalgia Rheumatica
69
Give examples of medium vessel vasculitic diseases? (2
- Polyarteritis nodosa
| - Kawasaki disease
70
Give an example of a small vessel vasculitic disease?
- Wegener's Granulomatosis
71
What is vasculitis?
- Inflammation of blood vessels
- Infarction of tissue:
- May present as oral inflammatory masses
- May present as ulcers (tissue necrosis)
The vessel wall thickens with inflammation
- Narrowing of lumen and reduced blood flow
72
In vasculitis there may be infarction of tissue. How can this present? (2)
- May present as oral inflammatory masses
| - May present as ulcers (tissue necrosis)
73
In vasculitis the vessel wall thickens with inflammation, what does this result in?
- Narrowing of lumen and reduced blood flow
74
What is Giant cell arteritis also known as?
- Known as 'temporal' arteritis as commonly the temporal artery is involved
75
What may giant cell arteritis present with?
- May present with headache/facial pain
76
Giant cell arteritis involves other carotid branches this can be known as 'chewing claudication'. What is this?
- Occlusion of central retinal artery (causes blindness)
| - IF we recognise this condition early and manage the inflammatory process we may be able to prevent this a bit)
77
Where does giant cell arteritis affect?
- This affects the distribution of the external carotid artery up the side of the head but also inside the face and heading in towards the central artery of the retina
78
Giant cell arteritis can cause a facial pain emergency. Can we test to confirm this is giant cell arteritis? (2)
- No specific test
- ESR (Erythrocyte sedimentation rate) is RAISED
- Also C-reactive protein & plasma viscosity
- 'Acute phase' reactants
- This suggests that the patient should be put on immunosuppressants such as prednisolone as early as possible
79
Polymyalgia Rheumatica is a disease of the elderly. What age group does it commonly affect and which part of the body does it commonly affect?
- Most aged 60-70 years
- Large blood vessels affected
- Is mainly seen around the shoulder and hip region and affects the muscles and joints of these areas
80
Polymyalgia Rheumatica often has some non-specific systemic features. Give examples of these? (3)
- Malaise
- Weight loss
- Profound fatigue
81
Which medication does polymyalgia rheumatica respond well to?
- Responds well to steroids - and takes only a very small dose of steroid to keep the condition settled
82
What are signs and symptoms of polymyalgia rheumatica? (7)
- Aches or pains in shoulders
- Aches or pain in neck, lower back, buttocks, hips or thighs
- Stiffness, especially in the morning
- Limited range of motion in affected joints
- Upper arm tenderness
- Less commonly, pain or stiffness in your wrists or knees
- Possible low grade fever early on, as well as fatigue, appetite loss, weight loss, depression
83
Which size of blood vessel does Kawasaki disease mainly affect?
- Medium blood vessels
84
Kawasaki disease is mainly a disease of children. give another name for it?
- Mucocutaneous lymph node syndrome
85
What are the clinical signs of Kawasaki disease? (4)
- Fever and lymphadenopathy
- Crusting/cracked tongue
- Strawberry tongue & erythematous mucosa
- Peeling rash on hands and feet
86
In Kawasaki disease, what can develop and what medication might need to be given?
- Coronary vessel aneurysm
| - They may need antibiotic cover but this would be determined by a cardiologist after an echocardiogram
87
Which size of blood vessels does Wegner's Granulomatosis affect?
- Small blood vessels
88
What is Wegner's Granulomatosis?
- It is an inflammatory condition
- Can lead to the destruction of hard and soft tissues of the face and oral cavity
- Spongy red tissue
89
Which systems will Wegner's granulomatosis affect?
- IT will affect many systems but often starts in the upper aerodigestive tract before spreading downwards into the lungs, the heart, the kidneys and the skin
- Renal and respiratory tract most affected
90
What is Wegner's granulomatosis associated with?
- Associated with ANCA - level correlates with clinical activity
91
What can we commonly see orally in a person with Wegner's granulomatosis?
- A very punctate looking vascular rash over the gingiva or the palate
- If we see this then urgent referral needs to be made for assessment
92
What is Fibromyalgia?
- Non-specific collection of musculoskeletal symptoms (joint pain, muscle pain, 'functional disorders')
- Diagnosis often used by rheumatologists when there are symptoms but without any evidence of active disease
- Probably more of a perceptual problem where the understanding of the pain from these areas is disrupted and the patient will genuinely have problems although there is no active disease present in the tissues
- This is a condition which is managed through pain management rather than immunosuppression
93
How is fibromyalgia managed?
- This condition is manages through pain management rather than immunosuppression
94
What are the dental aspects of Vasculitides? (2)
- Steroid precautions may be needed
May present to the dentist:
- Giant cell arteritis
- Wegner's Granulomatosis
- Kawasaki disease
95
Which vasculitis CTD's may present to the dentist? (3)
- Giant cell arteritis
- Wegner's granulomatosis
- Kawasaki disease
