giant cell (temporal) arteries Polymyalgia Rheumatica

Tissue changes without blood autoantibodies called ‘Discoid Lupus’ Seen in the skin and the mouth – looks similar to lichen planus

Connective Tissue Diseases Flashcards by niamh ritchie

2 groups of connective tissue diseases

autoimmune diseases (target tissues themselves)

vasculitic diseases (vessels are targets)

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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

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examples of autoimmune connective tissue diseases

Systemic lupus erythematosis (SLE)

Systemic sclerosis (Scleroderma)

Sjogrens syndrome (SS)

Undifferentiated connective tissue disease (UCTD)

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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

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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

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large vessel disease

giant cell (temporal) arteries

Polymyalgia Rheumatica

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medium vessel disease

polyarteritis nodosa

Kawasaki disease

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small vessel disease

Wegner’s Granulomatosis

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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

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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

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dicoid lupus

Tissue changes without blood autoantibodies called ‘Discoid Lupus’

Seen in the skin and the mouth – looks similar to lichen planus

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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

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systems affected in SLE

joints, skin, kidney, muscles, blood

CVS, RS & CNS

Renal invlovement previously major cause

Enhanced cardiovascular risk

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circulating immune complexes in SLE

ANA, dsDNA and Ro antibodies

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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

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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

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
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! ## Footnote *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 * 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