Connective Tissue Diseases

Question 1

What are the 4 multi system vasculitic inflammatory diseases?

Answer 1

• systemic lupus erythematosis (SLE)
• systemic sclerosis (scleroderma)
• Sjögren’s syndrome (SS)
• undifferentiated connective tissue disease (UCTD)

Question 2

What are the 5 vasculitic diseases and their categories?

Answer 2

• large vessel disease
  
  • giant cell (temporal) arteritis)
  • polymyalgia rheumatica
• medium vessel disease
  
  • polyarteritis nodosa
  • Kawasaki disease
• small vessel disease
  - Wegener’s granulomatosis

Question 3

How are connective tissue diseases generally managed?

Answer 3

• management similar for all diseases
• no cure as the cause of disease is not clear
• focus on suppression of disease activity instead
• analgesic NSAIDs
  
  • treatment of joint and muscle pain
• immune modulating treatment
  
  • hydroxychloroquine, methotrexate (first line)
  • azathioprine, mycophenolate (second line)
  • biological medication
    - cytokine inhibitors
    - synthetic antibodies (inflammatory/lymphocytic)
    - disrupt immune processes
• systemic steroids
  
  • short term option at any stage of disease
  • reduce inflammatory processes
  • control disease process
  • long term side effects prohibit long term and regular use

Question 4

What are all of the different lupus overlap diseases?

Answer 4

• lupus (every system involved + inflammatory organ disease)
• scleroderma
• Sjögren’s disease
• rheumatoid arthritis
• Raynaud’s phenomenon
• mixed connective tissue disease

Question 5

What antibodies are present in these autoimmune diseases?

Answer 5

• anti-nuclear antibody (ANA)
• anti-double-streand DNA (dsDNA)
• anti-Ro antibody (Ro)
• anti-La antibody (La)
• anti-centromere antibody
• anti-Scl-70 antibody
• anti-neutrophil cytoplasmic antibody (ANCA)
• top 4 most commonly found in most diseases
• ANA, dsDNA and Ro commonly found in overlap symptoms
• 5+6 associated with scleroderma

Question 6

What is discoid lupus?

Answer 6

• tissue changes without blood autoantibodies
  
  • localised to areas of skin and in the mouth
  • looks similar to lichen planus

Question 7

What is systemic lupus erythematosis and what immune complexes are likely present?

Answer 7

• changes to organs and the presence of autoantibodies
• ANA
  -dsDNA
  -Ro

Question 8

What does systemic lupus erythematosis affect?

Answer 8

• joints
• skin
• kidney
  
  • kidney involvement is major cause of death
• muscles
• blood
• cardiovascular
  
  • increased risk of acute cardiovascular disease
• central nervous system

Question 9

What are the symptoms of systemic lupus erythematosis?

Answer 9

• butterfly rash
  
  • photosensitive rash
• general photosensitivity
• Raynaud’s
  
  • associated with scleroderma

Question 10

What are the dental aspects of systemic lupus erythematosis?

Answer 10

• chronic anaemia
  
  • oral ulceration
  • increased risk with general anaesthetic
• thrombocytopenia
  
  • increased bleeding tendency
  • check platelets before extraction
• renal disease
  
  • impaired drug metabolism
• drug reactions
  
  • can trigger photosensitivity
• increased malignancy risk
  
  • steroid and immunosuppressant therapy
• oral mucosal reactions
  
  • looks like lichen planus
• oral pigmentation
  
  • hydroxychloroquine use

Question 11

What is the lupus anticoagulant?

Answer 11

• marker found in the blood of some patients with lupus
  
  • anticoagulants in a test tube but not in a patient
  • not true anticoagulant
  • indicates subtype of lupus patient

Question 12

What is antiphospholipid antibody syndrome?

Answer 12

• hypercoaguability due to thrombophilia
• antiphospholipid antibody syndrome
  
  • primary form
    - no other associated disease
  • secondary form
    - found in some patients with chronic inflammatory conditions
• antibodies
  
  • antiphospholipid (aPL)
  • anticardiolipin (aCL)
• characterised by recurrent thrombosis
  
  • DVT with pulmonary embolism
  • venous and arterial thrombosis
  • no other risk factors
• treated with anticoagulants

Question 13

What is Sjögren’s syndrome?

Answer 13

• inflammatory disease associated with circulating autoantibodies
  
  • ANA
  • Ro
  • La
• mainly associated with dry eyes and dry mouth
• can present as multi system disease
  
  • major involvement in salivary glands
  • other features of vasculitic immune disease

Question 14

What are the three main groups of Sjögren’s syndrome and what are their symptoms?

Answer 14

• sicca syndrome
  - dry eyes or dry mouth
• primary Sjögren’s
  
  • not associated with any other diseases
  • largely affecting mouth and eyes
• secondary Sjögren’s
  
  • associated with another connective tissue disease
  • often rheumatoid arthritis and systemic lupus erythematosis

Question 15

What is the diagnostic criteria for Sjögren’s syndrome?

Answer 15

• not possible through a simple test but some findings more important than others
• positive criteria for a diagnosis is helpful but usually comes down to clinical judgement
• dry eyes or mouth
• autoantibody findings
  
  • commonly ANA and Ro
• imaging findings
  
  • ultrasound and sialography
  • require disease to be at a high enough level to show change
• histopathological findings
  
  • inflammatory change evident
  • labial gland most reliable

Question 16

What are the dental aspects of Sjögren’s syndrome?

Answer 16

• oral infection
• caries risk
  
  • reduced salivary clearance
• functional loss
• denture retention
  
  • poor due to lack of saliva
• sialosis
  
  • swelling of salivary glands
• salivary lymphoma
  
  • as a result of chronic activation of lymphoid tissue
  • unilateral gland size change
  • usually after years

Question 17

What is systemic sclerosis?

Answer 17

• the replacement of elastic tissue with connective fibrous tissue
  
  • excessive collagen deposition
  • connective tissue fibrosis
• ability to store

Question 18

What are the two forms of systemic sclerosis and what antibodies are associated?

Answer 18

• local
  
  • anticentromere antibodies
• generalised
  
  • anti-Scl-70 antibodies

Question 19

What are the signs and symptoms of systemic sclerosis?

Answer 19

• ability to stretch and bend lost
  
  • sclerodactyly (contracture of fingers)
  • skin cannot stretch
• haemangiomas
  
  • evident on face and nails (telangiectasia and nail form capillaroscopy)
• loss of internal elastic tissue
  
  • less elastic oesophagus and GI tract
• ischaemic necrosis
  
  • occlusion of blood vessels
• Raynaud’s phenomenon
  
  • blood vessel patency narrowed
  • ischaemia turns tissue white
  • dilation turns tissue blue
  • vascular reperfusion turns tissue red
• renal failure
• malabsorption
• renal failure
• malabsorption

Question 20

What are the dental aspects of systemic sclerosis?

Answer 20

• involvement of perioral tissues
  
  • limited mouth opening
    - reduced elasticity of cheeks and perioral tissues
    - pulling on tissues can tear them
  • progressively poor oral access
    - restorative work, extractions, impressions not possible
  • limited tongue movement
• must treatment plan 10 years ahead
  
  • consider prognosis of all teeth
• dental problems compounded by disease
  
  • reduced salivary clearance
• dysphagia and reflect oesophagitis
  
  • swallowing difficulties
  • dental erosion
• cardiac and renal vasculitis disease
  
  • consider drug metabolism
• widening pf periodontal ligament space
  
  • no dental mobility

Question 21

What is vasculitis?

Answer 21

• inflammation of blood vessel walls
  
  • narrowing of vessels due to thickened wall
  • narrowed lumen reduces blood flow
  • infarction of tissues
    - oral inflammatory masses
    - ulcers (tissue necrosis)

Question 22

What is giant cell arteritis?

Answer 22

• vasculitis of the external carotid artery
  
  • affects many branches, known as temporal arteritis
  • causes chewing laudation
  • occlusion of dental retinal artery
    - ischaemic necrosis of the retina
    - leads to permanent blindness
• can present as headache or facial pain
• considered a facial pain emergency
  
  • no specific test
  • erythrocyte sedimentation rate (ESR) raised
  • C-reactive protein and plasma viscosity increased
  • immunosuppression (e.g. prednisolone) started as early as possible

Question 23

What is polymyalgia rheumatica?

Answer 23

• large vessel disease
• disease of the elderly
  
  • mostly 60-70 years
• pain and morning stiffness of muscles
  
  • shoulder and hips particularly
  • limited range of motion
• systemic features
  
  • malaise
  • weight loss
  • profound fatigue
• responds well to steroids
  
  • rapid response
  • only small dose required for management

Question 24

What is Kawasaki disease?

Answer 24

• medium vessel disease
• mainly affecting children
  
  • mucocutaneous lymph node syndrome
• clinical signs
  
  • fever and lymphadenopathy
  • crusting/cracked tongue
  • strawberry tongue/erythemous mucosa
  • peeling rash on hands and feet
• increased risk of coronary vessel aneurysms
  
  • may require antibiotic prescription
  • cardiologist involvement is a necessity

Question 25

What is Wegener’s granulomatosis?

Answer 25

• inflammatory condition of small blood vessels
  
  • destruction of hard and soft tissues
  • mainly in face and oral cavity
  • spongy red tissue
• associated with anti-neutrophil cytoplasmic antibody
  
  • level correlates with clinical activity
• starts in upper aerodigestive tract and travels down
  
  • renal and respiratory are worst affected
• most likely vasculitis condition to present to dentist
• vascular risk over gingiva and palate
  
  • urgent referral for assessment required

Question 26

What is fibromyalgia?

Answer 26

• non-specific collection of musculoskeletal symptoms
  
  • joint and muscle pain
  • symptoms without evidence of disease
  • likely perceptual problem
• managed by pain management
  
  • no immunosupression

Question 27

What are the dental aspects of vasculitis?

Answer 27

• steroid precautions may be needed
• most likely to present to dentist
  • giant cell arteritis
  • Wegener’s granulomatosis
  • Kawasaki disease