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BDS3 BAMS Diseases of Human Systems > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

2 groups of connective tissue diseases

A

autoimmune diseases (target tissues themselves)

vasculitic diseases (vessels are targets)

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2
Q

autoimmune connective tissue diseases

A
  • 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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3
Q

examples of autoimmune connective tissue diseases

A

Systemic lupus erythematosis (SLE)

Systemic sclerosis (Scleroderma)

Sjogrens syndrome (SS)

Undifferentiated connective tissue disease (UCTD)

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4
Q

features of autoimmune multisytem vasculitic diseases

A
  • 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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5
Q

vasculitis connective tissue diseases

3 categories

A
  • Large vessel Disease
    • Giant cell (temporal) arteritis
  • Medium Vessel Disease
    • Polyarteritis nodosa
    • Kawasaki disease
  • Small vessel Disease
    • Wegener’s Granulomatosis
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6
Q

large vessel disease

A

giant cell (temporal) arteries

  • Polymyalgia Rheumatica
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7
Q

medium vessel disease

A

polyarteritis nodosa

Kawasaki disease

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8
Q

small vessel disease

A

Wegner’s Granulomatosis

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9
Q

general management of connective tissue disease

A
  • 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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10
Q

immune connective tissue diseases are

A

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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11
Q

dicoid lupus

A

Tissue changes without blood autoantibodies called ‘Discoid Lupus’

  • Seen in the skin and the mouth – looks similar to lichen planus
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12
Q

systemic lupus erythematosis

A

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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13
Q

systems affected in SLE

A

joints, skin, kidney, muscles, blood

CVS, RS & CNS

Renal invlovement previously major cause

Enhanced cardiovascular risk

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14
Q

circulating immune complexes in SLE

A

ANA, dsDNA and Ro antibodies

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15
Q

prevalance of SLE

A
  • 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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16
Q

dental aspects of SLE (7)

A
  • 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
17
Q

lupus anticoagulant

A

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

18
Q

Antiphospholipid Antibody Syndrome (APS)

A
  • Characterised by recurrent Thrombosis
    • DVT with pulmonary embolism
  • Venous & arterial thrombosis
    • Patients anticoagulated
  • commonly on warfarin
    • Do not stop anticoagulant – need physician advice
19
Q

Sjögren’s syndrome

A
  • 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
20
Q

3 main groups of sjögren’s

A
  • 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.
21
Q

sjörgen’s syndrome diagnostic criteria

A

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

22
Q

Sicca syndrome sjögren’s

A

dry eyes or dry mouth

23
Q

primary sjögren’s

A

not associated with any other disease

24
Q

secondary sjögren’s

A

associated with another connective tissue disease - RA, SLE etc

25
Q

Sjögren’s syndron - oral and dental implications

A
  • 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
26
Q

systemic sclerosis

A
  • 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
27
Q

systemic sclerosis prevalence

A
  • predominantly women
  • gradual onset
    • Raynoud’s phenomenon
    • renal failure
    • Malabsorbtion (GI involvement)
  • slow progression
    • no treatment to prevent
28
Q

dental aspects of systemic sclerosis

A
  • 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
29
Q

vasculitis

A
  • 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
30
Q

giant cell arteritis

A
  • 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
31
Q

polymtalgia rheumatica

A

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

32
Q

Kawasaki disease

A
  • 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

33
Q

Wegner’s Granulomatosis

A

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
34
Q

fibromyalgia

A

symptoms but no evidence of active disease

  • Non-specific collection of musculoskeletal symptoms
    • Joint pain
    • Muscle pain
    • ‘functional disorders’

Pain management rather than immunosuppression

35
Q

dental aspects of vasculitides

A
  • Steroid precautions may be needed
  • May present to the dentist
    • Giant cell arteritis
    • Wegener’s Granulomatosis
    • Kawasaki Disease

BDS3 BAMS Diseases of Human Systems (13 decks)

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