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Rheumatology > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What is the pathogenesis of systemic lupus erythematosis (SLE)?

A

Defective apoptosis increases cell death and releases auto-antigens which form immune complexes. These deposit in small blood vessels, skin, kidneys and other places causing wide range of problems across the body

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2
Q

How does systemic lupus erythematosis (SLE) present?

A

Wide ranging presentation as it affects multiple body systems.

Fever, fatigue, weight loss

Myalgia, arthalgia

Malar rash, alopecia, photosensitivity

Glomerulonephritis

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3
Q

What may a FBC show in systemic lupus erythematosis (SLE)?

A

Low RBC, (anaemia)

Low WBC ( leukopenia)

Low platelets (thrombocytopenia)

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4
Q

What markers can be used to assess SLE disease activity?

A

Complement (low in active disease as it is used up)

Anti-dsDNA (high in active disease)

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5
Q

What autoantibodies are associated with systemic lupus erythematosis (SLE)?

What are the specificities and sensitivities of these auto-antibodies?

A

Anti-nuclear antibody (ANA)
Anti-double stranded (anti-dsDNA)

ANA

  • high sensitivity (95% SLE patients positive)
  • non-specific (1/5 general people positive)

If ANA negative, they don’t have SLE. If its positive, doesn’t mean they definitely have SLE.

Anti-dsDNA

  • moderate specificity (60% SLE patents positive)
  • high specificity

If anti-dsDNA positive, they definitely have SLE. If its negative doesn’t mean they definitely don’t have SLE

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6
Q

What drug are most patients with systemic lupus erythematosis (SLE) on?

A

Hydroxychloroquine

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7
Q

What is used in the treatment of mild, moderate and severe systemic lupus erythematosis (SLE)?

A

Mild

  • hydroxochloroquine
  • topical steroids (low dose <15mg/day)

Moderate

  • DMARDs (methotrexate, azathioprine)
  • oral steroids (moderate dose 0.5mg/day)

Severe

  • cyclophosphamide
  • IV steroids
  • biologics if resistance (ritixumab)
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8
Q

What is the most important investigation to do if systemic lupus erythematosis (SLE) is suspected/confirmed and what are you looking for?

A

Urinalysis to see if renal involvement

Proteinuria/heamaturia would suggest lupus nephritis

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9
Q

What tests are used to monitor systemic lupus erythematosis (SLE)?

A

Measuring complement and anti-dsDNA (assesses disease activity)

Urinalysis (screen for lupus nephritis)

Blood pressure and cholesterol (assess cardiovascular risk factors, increased risk of CVD)

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10
Q

What is Sjogren’s syndrome?

A

Autoimmune condition causing disruption of lacrimal and salivary glands due to lymphocytic infiltration

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11
Q

How does Sjogren’s syndrome present?

A

Fatigue

Dry eyes, mouth, vagina

Parotid swelling

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12
Q

What clinical test can be useful in diagnosing Sjogren’s syndrome? What does it show?

A

Schirmer’s test
Assesses ocular dryness

Place filter paper in eyes and see how much water drains into paper (>5mm in 5 mins is positive)

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13
Q

What auto-antibodies are associated with Sjogren’s syndrome?

A

Anti-Ro

Anti-La

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14
Q

What is the general treatment of Sjogren’s syndrome?

A

Symptomatic eye drops and salivary replacement drops

Hydroxychloroquine for fatigue/joint pain

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15
Q

In what circumstances would methotrexate be indicated in the treatment of Sjogren’s syndrome?

A

Organ involvement (e.g. interstitial lung disease)

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16
Q

What can Sjogren’s syndrome occur secondary to?

A 
Rheumatoid arthritis (RA)
Systemic lupus erythematosis (SLE)
17
Q

What is systemic sclerosis?

A

Autoimmune disorder characterised by increased collagen production causing skin tightening, Raynaud’s phenomenon and some organ dysfunction

18
Q

What skin changes can be seen in systemic sclerosis?

A

Nose beaking
Tight skin around mouth
Sclerodactyly

19
Q

What is the progression of skin damage in systemic sclerosis?

A
  1. Oedematous
  2. Indurative (hardening)
  3. Atrophic
20
Q

What are the major and minor features of systemic sclerosis?

How does this help diagnosis?

A

Major - sclerosis of the skin of face, neck and arms

Minor - sclerodactyly, fingertip atrophy, bilateral lung fibrosis

Systemic sclerosis diagnosis confirmed with 1 major cutaneous feature and 2 minor cutaneous features

21
Q

What organs can be affected by systemic sclerosis?

A

Lungs (bilateral lung fibrosis)

Oesophagus (osophageal dysmotility causing acid reflux)

22
Q

What is the difference between limited and diffuse systemic sclerosis?

A

Limited systemic sclerosis

  • skin of face, arms and hands/feet involved
  • organ involvement occurs late

Diffuse systemic sclerosis

  • skin of trunk involved (rapid development)
  • organ involvement occurs early
23
Q

What autoantibodies are associated with systemic sclerosis?

Which is/are more common in limited and diffuse systemic sclerosis?

A

Anti-centromere (associated with limited)

Anti-Scl-70 (associated with diffuse)

24
Q

What is the treatment of systemic sclerosis?

A

No specific management

Management tailored towards specific issues

25
Q

What treatment can be used for Raynaud’s phenomenon?

A

Calcium channel blocker (amlodipine)

26
Q

What treatment can be used for digital ulcers secondary to systemic sclerosis?

A

Iloprost infusion

27
Q

What is anti-phospholipid syndrome (APS)?

A

Autoimmune disorder characterised by recurrent thrombosis

28
Q

How does anti-phospholipid syndrome (APS) present?

A

Increased incidence of strokes, MI (possibly in young patient)

Foetal loss

Migraine

Levido reticularis (lace-like purple discolouration of skin)

29
Q

What is catastrophic anti-phospholipid syndrome (CAPS)

A

Rare but deadly (50% mortality) form of anti-phospholipid syndrome causing multi-organ infarction over days/weeks

30
Q

What antibodies are associated with anti-phospholipid syndrome (APS)?

A

Anti-cardiolipin
Anti-ß2-glycoprotein
Lupus anticoagulant

31
Q

What happens to the partial thromboplastin time (PTT) in anti-phospholipid syndrome (APS)?

A

Paradoxically prolonged

32
Q

How is the treatment of anti-phospholipid syndrome (APS)?

A

If they have had thrombotic episode, lifelong warfarin

If they have had recurrent pregnancy loss, life long LWMH (warfarin is teratogenic)

33
Q

What is the management of a patient who has not had a thrombotic episode but is positive for all the antibodies associated with anti-phospholipid syndrome (APS)?

A

Nothing, they don’t require anticoagulation

34
Q

What is a positive result for anti-phospholipid syndrome (APS) autoantibodies?

A

Positive on 2 occasions 12 weeks apart

35
Q

What is mixed connective tissue disease?

A

Defined condition characterised by features of other autoimmune connective tissue disorders

36
Q

What autoantibodies are associated with mixed connective tissue disease?

A

Anti-RNP

Rheumatology (7 decks)

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