Connective Tissue Diseases Flashcards
2 groups of connective tissue diseases
autoimmune diseases (target tissues themselves)
vasculitic diseases (vessels are targets)
autoimmune connective tissue diseases
- Multisystem vasculitic inflammatory diseases:
- Systemic lupus erythematosis (SLE)
- Systemic sclerosis (Scleroderma)
- Sjogrens syndrome (SS)
- Undifferentiated connective tissue disease (UCTD)
- Have associated blood autoantibodies
- DO NOT cause the disease – are found in ‘normal’ people
- Pattern varies from disease to disease
Complement activation causes tissue damage
examples of autoimmune connective tissue diseases
Systemic lupus erythematosis (SLE)
Systemic sclerosis (Scleroderma)
Sjogrens syndrome (SS)
Undifferentiated connective tissue disease (UCTD)
features of autoimmune multisytem vasculitic diseases
- Have associated blood autoantibodies
- DO NOT cause the disease – are found in ‘normal’ people
- Pattern varies from disease to disease
- Complement activation causes tissue damage
vasculitis connective tissue diseases
3 categories
- Large vessel Disease
- Giant cell (temporal) arteritis
- Medium Vessel Disease
- Polyarteritis nodosa
- Kawasaki disease
- Small vessel Disease
- Wegener’s Granulomatosis
large vessel disease
giant cell (temporal) arteries
- Polymyalgia Rheumatica
medium vessel disease
polyarteritis nodosa
Kawasaki disease
small vessel disease
Wegner’s Granulomatosis
general management of connective tissue disease
- Dependant on disease activity
- Immune suppression – no cure as cause unclear
- Analgesic NSAIDs (joint/muscle symptoms)
- Immune modulating treatment
- Hydroxychloroquine
- Methotrexate
- Azathioprine
- Mycophenolate
- Biologic Medication – Cytokine inhibitors and lymphocyte depleting drugs
- Systemic steroids - prednisolone
immune connective tissue diseases are
interlinked
features overlap
pt presentation unique
Antibodies in Autoimmune diseases
- Anti-nuclear antibody (ANA)
- Anti-Ro antibody (Ro)
- Anti-La antibody (La)
overlap
- Anti-Scl-70 antibody
- Anti-Centromere antibody
- Anti-Neutrophil Cytoplasmic Antibody (ANCA)
last three sclerodoma
dicoid lupus
Tissue changes without blood autoantibodies called ‘Discoid Lupus’
- Seen in the skin and the mouth – looks similar to lichen planus
systemic lupus erythematosis
blood antibody changes
- Circulating immune complexes
- ANA, dsDNA & Ro antibodies
Multi-system
- joints, skin, kidney, muscles, blood
- CVS, RS & CNS
- Renal invlovement previously major cause
- Enhanced cardiovascular risk
12-64/100,000 population
Twins 20-50% concordance
- Siblings same as non-relatives 5%
females of child bearing age
photosensitivity common
- Butterfly zygomatic rash
- Generally avoid sun
systems affected in SLE
joints, skin, kidney, muscles, blood
CVS, RS & CNS
Renal invlovement previously major cause
Enhanced cardiovascular risk
circulating immune complexes in SLE
ANA, dsDNA and Ro antibodies
prevalance of SLE
- 12-64/100,000 population
- Twins 20-50% concordance
- Siblings same as non-relatives 5%
- females of child bearing age
photosensitive - avoid sun
dental aspects of SLE (7)
- Chronic anaemia
- oral ulceration
- GA risk
- Bleeding tendency
- Thrombocytopenia – bleeding tendency on extraction
- renal disease
- impaired drug metabolism
- drug reactions – photosensitivity
- steroid & immunosuppresive therapy
- Possible increased malignancy risk
- lichenoid oral reactions
- oral pigmentation from hydroxychloroquine use
lupus anticoagulant
Marker found in the blood of some patients with Lupus
- anticoagulates blood in a test tube but NOT in a patient
Indicates a ‘subtype’ of lupus patient
- These patients termed “antiphospholipid antibody syndrome”
- Primary Form
- No other associated disease
- Secondary Form
- Found in some patients with chronic inflammatory conditions e.g. SLE
- Primary Form
Antibodies
- Antiphospholipid (aPL)
- Anticardiolipin (aCL)
Can be primary – no other diseases associated with the antiphopholipid syndrome or secondary – lupus, sjörgen’s
Antiphospholipid Antibody Syndrome (APS)
- Characterised by recurrent Thrombosis
- DVT with pulmonary embolism
- Venous & arterial thrombosis
- Patients anticoagulated
- commonly on warfarin
- Do not stop anticoagulant – need physician advice
Sjögren’s syndrome
- Inflammatory disease ASSOCIATED with circulating autoantibodies
- ANA, Ro and La
- Mainly associated with dry eyes and dry mouth
- Multisystem in some
- Major involvement in Salivary glands
- Leads to Xerostomia and oral disease
3 main groups of sjögren’s
- Sicca Syndrome
- Dry eyes or Dry mouth
- Primary Sjögren’s
- Not associated with any other disease
- Secondary Sjögren’s
- Associated with another Connective Tissue disease – Rheumatoid Arthiritis, SLE, etc.
sjörgen’s syndrome diagnostic criteria
No one test that ‘proves’ Sjögren’s syndrome
Some signs/symptoms more important than others
- Dry eyes/mouth
- Subjective or objective
- Lacrimal and salivary glands changes can take 30 years to show clinically due to residual capacity– need histological exam to test properly
- Subjective or objective
- Autoantibody findings
- Imaging findings
- Histopathology findings
Positive criteria for diagnosis often a clinical judgement
Sicca syndrome sjögren’s
dry eyes or dry mouth
primary sjögren’s
not associated with any other disease
secondary sjögren’s
associated with another connective tissue disease - RA, SLE etc
Sjögren’s syndron - oral and dental implications
- Oral Infection
- Caries risk
- Functional loss
- Denture retention
- Sialosis – swelling salivary gland
- Salivary lymphoma – result of chronic activation of lymphatic tissue
- Unilateral gland size change
- usually after years
systemic sclerosis
- excessive collagen deposition
- connective tissue fibrosis
- loss of elastic tissue
- local or generalised forms
- Local - anticentromere antibodies
- Generalised – anti Scl-70 antibodies
issues
- sclerodactyly
- raynaud’s
- telangiectasia
- nailfold capillaroscopy
Many hidden problems
- Oesophagus lack stretch
- Intestine – peristaltic issues
systemic sclerosis prevalence
- predominantly women
- gradual onset
- Raynoud’s phenomenon
- renal failure
- Malabsorbtion (GI involvement)
- slow progression
- no treatment to prevent
dental aspects of systemic sclerosis
- involvement of perioral tissues
- limited mouth opening – lack elasticity of tissues
- progressively poor oral access
- limited tongue movement
- PLAN TREATMENT 10yrs AHEAD!
- May be compounded by sjogrens!
- dysphagia & reflux oesophagitis
- Swallowing difficulties
- Dental erosion
- Cardiac and renal vasculitic disease
- Watch drug metabilusm
- Widening of PDL space
- No dental mobility
vasculitis
- inflammation of blood vessels
- infarction of tissue
- May present as oral inflammatory masses
- May present as ulcers (tissue necrosis)
- Vessel wall thickens with inflammation
- Narrowing of lumen reduced blood flow
giant cell arteritis
- Known as ‘temporal’ arteritis as commonly the Temporal Artery is involved
- Distribution of ECA
- May present with Headache/facial pain
- Involves other carotid branches
- “Chewing claudication”
- Occlusion/narrowing of central retinal artery (blindness)
A facial pain emergency! – need managed if suspected
- No specific test
- ESR (Erythrocyte sedimentation rate) is RAISED
- Also C-reactive protein & plasma viscosity
- “Acute Phase” reactants
polymtalgia rheumatica
pain and aches in shoulder and hip regions, morning worse
- Disease of the elderly
- Most aged 60-70 years
- Large blood vessels affected
- Pain & morning stiffness of muscles
- Non-specific systemic features
- Malaise, weight loss, profound fatigue
Responds well to steroids – only need small dose
Kawasaki disease
- Mainly a disease of Children
- AKA “mucocutaneous lymph node syndrome”
- Clinically gives
- Fever & lymphadenopathy
- Crusting/cracked tongue
- Strawberry tongue & erythematous mucosa
- Peeling rash and erythema on hands and feet
- Coronary vessel aneurysms
- May need antibiotic cover – see cardiologist!
Imp to recognise erythematous tongue and crusty lips and consider Kawasaki
Wegner’s Granulomatosis
most likely present to dentist
- Inflammatory condition
- Many systems – start in URT
- Spread to lungs, heart, kidneys
- Can lead to destruction of hard and soft tissues of the face and oral cavity
- Spongy red tissue – urgent referral for assessment
- Many systems – start in URT
- Associated with ANCA
- Level correlates with clinical activity
- Renal and respiratory tract most affected
fibromyalgia
symptoms but no evidence of active disease
- Non-specific collection of musculoskeletal symptoms
- Joint pain
- Muscle pain
- ‘functional disorders’
Pain management rather than immunosuppression
dental aspects of vasculitides
- Steroid precautions may be needed
- May present to the dentist
- Giant cell arteritis
- Wegener’s Granulomatosis
- Kawasaki Disease
