Connective Tissue Diseases (R) Flashcards

1
Q

What can connective tissue disease be broken down into

A

2 groups
autoimmune diseases
vasculitic diseases

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2
Q

What are connective tissue autoimmune diseases

A

multisystem vasculitic inflammatory diseases

The tissues themselves are inflamed (whereas in vasculitic the vessels are the target of inflammation)

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3
Q

What are the 4 CT autoimmune diseases

A

systemic lupus erythematosis
systemic sclerosis
sjrogens syndrome SS
undifferentiated connective tissue disease

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4
Q

What do the autoimmune diseases have in common

A

they have associated blood antibodies

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5
Q

Do the antibodies cause the disease

A

no, they are found in normal people and the pattern varies from disease to disease

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6
Q

What causes the tissue damage in the autoimmune diseases

A

complement activation

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7
Q

What are examples of vasculitic diseases

A

large vessel disease
medium vessel disease
small vessel disease

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8
Q

What is an example of large vessel disease

A

giant cell (temporal) arteritis

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9
Q

What are examples of medium vessel disease

A

polyartertitis nodosa

kawasaki disease

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10
Q

What is an example of smalll vessel disease

A

wegener’s granulomatosis

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11
Q

What is the general management for connective tissue disease

A

dependent on disease activity
analgesic NSAIDs (for joint/muscle symtoms)
immune modulating treatment
systemic steroids

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12
Q

What are analgesic NSAIDs given for?

A

for joint and muscle symptoms

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13
Q

What are the possible immune modulating treatment

A
hydroxychloroquine 
methotrexate 
azathioprine
mycophenolate
biologic medications
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14
Q

What are biologic medications

A

these can be used if immune modulators are not enough and they are synthetic antibodies against certain inflammatory markers or lymphocytic markers

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15
Q

What systemic steroid is used in connective tissue disease management and what can it be used for?

A

prednisolone
can be used at any stage short term to reduce the inflammatory process but due to their long term side effects they are not used regularly

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16
Q

Describe lupus

A

lupus has every system involved
it can have features of any of the other diseases as well as inflammatory organ diseases (this part is characteristic to lupus)

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17
Q

What are the antibodies that are commonly found in connective tissue disease

A

anti-nuclear antibody (ANA)
anti-double strand DNA (dsDNA)
anti-Ro antibody (Ro)
anti-la antibody (La)

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18
Q

What antibody is commonly found in scleroderma

A

anti-centromere antibody
anti-scl-70 antibody
anti neutrophil cytoplasmic antibody (ANCA)

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19
Q

What is systemic lupus erythematosis?

A

tissue changes without blood autoantibodies called ‘discoid lupus’ (because you get discoid lupus and systemic lupus erythematosis)

seen in the skin and mouth and looks similar to lichen planes

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20
Q

What are the circulating immune complexes with systemic lupus erythematosis

A

ANA
dsDNA
Ro antibodies

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21
Q

What are the systems effected by SLE

A

joints, skin, kidney, muscles, blood
CVS, RS, CNS
renal involvement previously major cause
enhanced cardiovascular risk

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22
Q

What are the features of SLE

A

particularly genetic - seen in twins
environmental trigger
females of child bearing age
may see a photosensitive rash

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23
Q

What are dental aspects of SLE

A

chronic anaemia (GA risk and oral ulceration)
bleeding tendency (thrombocytopenia - need platelets checked before an extraction)
renal disease (impaired drug metabolism)
drug reactions - can trigger photosensitivity
steroid and immunosuppressive therapy
lichenoid oral reactions
oral pigmentation from hydroxychloroquine use

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24
Q

What is a lupus anticoagulant?

A

it is a marker founds in the blood of some px with lupus
it is not a true anticoagulant but indicates a subtype of lupus px (it anticoagulates blood in a test tube but NOT in the patient)

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25
Q

What is the subtype of px with lupus anticoagulant called

A

antiphospholipid antibody syndrome

26
Q

What is the primary form of antiphospholipid antibody syndrome

A

it has no other associated disease

27
Q

What is the secondary form of antiphospholipid syndrome

A

found in some patients with chronic inflammatory conditions such as SLE

28
Q

What antibodies are seen in the antiphospholipid antibody syndrome

A

antiphospholipid (aPL)

anticardiolipin (aCl)

29
Q

What is antiphospholipid antibody syndrome characterized by

A

-recurrent thrombosis (DVT with pulmonary embolism)

are at risk of venous and arterial thrombosis so usually require anticoagulant

Note: DO NOT STOP the patients anticoagulant without talking to the physician

30
Q

What is sjogrens syndrome

A

inflammatory disease associated with (but not caused by) circulating autoantibodies (ANA, Ro and La)

31
Q

What symptoms are mainly associated with sjogrens

A

dry eyes
dry mouth

can be multisystem

32
Q

What is sicca syndrome in sjogrens

A

dry eyes dry mouth

33
Q

What is primary sjogrens

A

not associated with any other disease

34
Q

What is secondary sjogrens

A

associated with another connective tissue disease e.g RA or SLE

35
Q

What is the diagnostic criteria for sjogrens

A

no one test that proves it
some signs and symptoms are more important than other
Look at dry eyes/mouth, autoantibody findings
imaging findins
histopathology findings
positive criteria for diagnosis often a clinical judgement

36
Q

What are the oral and dental implications of sjogrens

A
oral infection
caries risk
functional loss
denture retention 
sialosis
salivary lymphoma - unilateral gland size change - usually after years
37
Q

What is systemic sclerosis

A

excessive collagen deposition
connective tissue fibrosis
loss of elastic tissue
local or generalized forms

38
Q

What antibody is associated with local systemic sclerosis

A

anticentromere antibodies

39
Q

What antibody is associated with general systemic sclerosis

A

anti Scl-70 antibodies

40
Q

What gender predominantly suffer SS

A

women

41
Q

What is the gradual onset of systemic sclerosis

A

raynoud’s phenomenon
then renal failure due to vasculitic damage
malabsorption as there is GI involvement
slow progression - no treatment to prevent

42
Q

What is the dental aspect of systemic sclerosis

A
involvement of perioral tissues
provide provision for patient ahead (slow progression)
may be compounded by sjrogens 
dysphagia and reflux oesophagitis
cardiac and renal vasculitic disease
widening of periodontal ligament space
43
Q

What does the involvement of perioral tissues in Ss result in

A

limited mouth opening
progressively poor oral access
limited tongue movements

44
Q

What does dysphagia and reflux esophagitis result in

A

swallowing difficulties

dental erosion

45
Q

What is significant about the widening of the PDL in SS

A

no dental mobility

46
Q

What is vasculitis

A

inflammation of blood vessels
can result in infraction of tissue
vessel wall thickens with inflammation narrowing the lumen & reducing BF

47
Q

How may the infarction of tissue present in vasculitis

A

oral inflammatory masses

ulcers (tissue necrosis)

48
Q

What is giant cell arteritis

A

known as temporal arteritis as commonly the temporal artery is involved
involves other carotid branches

49
Q

How can giant cell arteritis present

A

headache/facial pain

50
Q

What can involvement of carotid branches in GCA result in

A

chewing claudication

occlusion of central retinal artery - blindness

51
Q

If someone comes in with facial pain emergency what can be tested to see if GCA is expected

A

Erythrocyte sedimentation rate is raised
c-reactive protein and plasma viscosity
acute phase reactants

52
Q

What is polymayalgia rheumatic

A

disease of the elderly

large BV effected

53
Q

What are symptoms of polymyalgia rheumatica

A

pain and morning stiffness of muscles
non specific systemic features e.g malaise and weight loss

responds well to steroids

54
Q

What is kawasaki disease

A

mainly a disease of children

55
Q

What does kawasaki disease clinically give

A
fever and lymphadenopathy
crusting/cracked tongue
strawberry tongue & erythematous mucosa
peeling rash on hands and feet
coronary vessel aneurysms - may need Ab cover
56
Q

What is wegener granulomatosis

A

inflammatory condition that can lead to destruction of hard and soft size fo the face and oral cavity giving look of spongy red tissue

57
Q

What is wegener granulomatosis associated with

A

ACNA

level correlates with clinical activity

58
Q

What is most affected in WG

A

renal and resp tract

59
Q

What is fibromyalgia

A

non specific collection of musculoskeletal symptoms

60
Q

What are the symptoms of FM

A

joint pain

muscle pain

61
Q

What are the dental aspects of vasculitides

A

steroid precaution may be needed

diseases that may present to the dentist are GCA, WG and kawasaki disease