Connective Tissue Diseases (R)

Question 1

What can connective tissue disease be broken down into

Answer 1

2 groups
autoimmune diseases
vasculitic diseases

Question 2

What are connective tissue autoimmune diseases

Answer 2

multisystem vasculitic inflammatory diseases

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

Question 3

What are the 4 CT autoimmune diseases

Answer 3

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

Question 4

What do the autoimmune diseases have in common

Answer 4

they have associated blood antibodies

Question 5

Do the antibodies cause the disease

Answer 5

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

Question 6

What causes the tissue damage in the autoimmune diseases

Answer 6

complement activation

Question 7

What are examples of vasculitic diseases

Answer 7

large vessel disease
medium vessel disease
small vessel disease

Question 8

What is an example of large vessel disease

Answer 8

giant cell (temporal) arteritis

Question 9

What are examples of medium vessel disease

Answer 9

polyartertitis nodosa

kawasaki disease

Question 10

What is an example of smalll vessel disease

Answer 10

wegener’s granulomatosis

Question 11

What is the general management for connective tissue disease

Answer 11

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

Question 12

What are analgesic NSAIDs given for?

Answer 12

for joint and muscle symptoms

Question 13

What are the possible immune modulating treatment

Answer 13

hydroxychloroquine 
methotrexate 
azathioprine
mycophenolate
biologic medications

Question 14

What are biologic medications

Answer 14

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

Question 15

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

Answer 15

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

Question 16

Describe lupus

Answer 16

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)

Question 17

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

Answer 17

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

Question 18

What antibody is commonly found in scleroderma

Answer 18

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

Question 19

What is systemic lupus erythematosis?

Answer 19

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

Question 20

What are the circulating immune complexes with systemic lupus erythematosis

Answer 20

ANA
dsDNA
Ro antibodies

Question 21

What are the systems effected by SLE

Answer 21

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

Question 22

What are the features of SLE

Answer 22

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

Question 23

What are dental aspects of SLE

Answer 23

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

Question 24

What is a lupus anticoagulant?

Answer 24

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)

Question 25

What is the subtype of px with lupus anticoagulant called

Answer 25

antiphospholipid antibody syndrome

Question 26

What is the primary form of antiphospholipid antibody syndrome

Answer 26

it has no other associated disease

Question 27

What is the secondary form of antiphospholipid syndrome

Answer 27

found in some patients with chronic inflammatory conditions such as SLE

Question 28

What antibodies are seen in the antiphospholipid antibody syndrome

Answer 28

antiphospholipid (aPL) | anticardiolipin (aCl)

Question 29

What is antiphospholipid antibody syndrome characterized by

Answer 29

-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

Question 30

What is sjogrens syndrome

Answer 30

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

Question 31

What symptoms are mainly associated with sjogrens

Answer 31

dry eyes dry mouth can be multisystem

Question 32

What is sicca syndrome in sjogrens

Answer 32

dry eyes dry mouth

Question 33

What is primary sjogrens

Answer 33

not associated with any other disease

Question 34

What is secondary sjogrens

Answer 34

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

Question 35

What is the diagnostic criteria for sjogrens

Answer 35

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

Question 36

What are the oral and dental implications of sjogrens

Answer 36

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

Question 37

What is systemic sclerosis

Answer 37

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

Question 38

What antibody is associated with local systemic sclerosis

Answer 38

anticentromere antibodies

Question 39

What antibody is associated with general systemic sclerosis

Answer 39

anti Scl-70 antibodies

Question 40

What gender predominantly suffer SS

Answer 40

women

Question 41

What is the gradual onset of systemic sclerosis

Answer 41

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

Question 42

What is the dental aspect of systemic sclerosis

Answer 42

``` 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 ```

Question 43

What does the involvement of perioral tissues in Ss result in

Answer 43

limited mouth opening progressively poor oral access limited tongue movements

Question 44

What does dysphagia and reflux esophagitis result in

Answer 44

swallowing difficulties | dental erosion

Question 45

What is significant about the widening of the PDL in SS

Answer 45

no dental mobility

Question 46

What is vasculitis

Answer 46

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

Question 47

How may the infarction of tissue present in vasculitis

Answer 47

oral inflammatory masses | ulcers (tissue necrosis)

Question 48

What is giant cell arteritis

Answer 48

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

Question 49

How can giant cell arteritis present

Answer 49

headache/facial pain

Question 50

What can involvement of carotid branches in GCA result in

Answer 50

chewing claudication | occlusion of central retinal artery - blindness

Question 51

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

Answer 51

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

Question 52

What is polymayalgia rheumatic

Answer 52

disease of the elderly | large BV effected

Question 53

What are symptoms of polymyalgia rheumatica

Answer 53

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

Question 54

What is kawasaki disease

Answer 54

mainly a disease of children

Question 55

What does kawasaki disease clinically give

Answer 55

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

Question 56

What is wegener granulomatosis

Answer 56

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

Question 57

What is wegener granulomatosis associated with

Answer 57

ACNA | level correlates with clinical activity

Question 58

What is most affected in WG

Answer 58

renal and resp tract

Question 59

What is fibromyalgia

Answer 59

non specific collection of musculoskeletal symptoms

Question 60

What are the symptoms of FM

Answer 60

joint pain | muscle pain

Question 61

What are the dental aspects of vasculitides

Answer 61

steroid precaution may be needed | diseases that may present to the dentist are GCA, WG and kawasaki disease