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BRS 2 > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

What is rheumatoid arthritis?

A

Chronic joint inflammation can result in joint damage

Site of inflammation = synovium

Associated with autoantibodies (RF and Anti-CCP)

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2
Q

Which autoantibodies are associated with rheumatoid arthritis?

A

Rheumatoid factor

Anti-CCP

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3
Q

What is the site of inflammation for rheumatoid arthritis?

A

Synovium

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4
Q

What is ankylosing spondylitis?

A
  • Chronic spinal inflammation that can result in spinal fusion and deformity- spinal fusion is detected on the radiograph as a ‘Bamboo spine’.
  • Site of inflammation includes the enthesis
  • No autoantibodies (Seronegative).
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5
Q

What are the consequences with ankylosing spondylitis?

A

Spinal fusion

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6
Q

How is spinal fusion evidently seen on a radiograph?

A

Bamboo spine

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7
Q

What are the examples of seronegative spondyloarthopathies?

A
  • Ankylosing spondylitis
  • Reactive arthritis (Reiter’s Syndrome)
  • Arthritis associated with psoriasis (Psoriatic arthritis)
  • Arthritis associated with gastrointestinal inflammation (enterohepatic synovitis).
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8
Q

What is SLE?

A
  • Chronic tissue inflammation in the presence of antibodies directed against self-antigens.
  • Multi-site inflammation – predominantly in the joints, skin and kidney.
  • The formation of immune complexes  Type III hypersensitivity.
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9
Q

What type of hypersensitivity is associated with SLE?

A

Type 3 hypersensitivity -due to formation of immune complexes

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10
Q

Which three main autoantibodies are associated with SLE?

A

Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies

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11
Q

Which typeof rash is associated with SLE?

A

Malar rash

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12
Q

Which sex is mainly affected by SLE?

A

Female

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13
Q

In which age group is SLE is diagnosed typically?

A

15-45 years

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14
Q

What are the key features of SLE?

A

Arthralgias and arthritis are typically non-erosive

Serum autoantibodies are characteristic

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15
Q

What is Raynaud’s phenomenn?

A

Intermittent vasospasm of digits on exposure to cold

1) Vasospasm - blanching of the digit
2) Cyanosis
3) Reactive hyperaemia

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16
Q

What are the three stages of Raynaud’s?

A

1) Vasospasm
2) Cyanosis
3) Reactie hyperaemia

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17
Q

What are the colour changes seen in Raynaud’s?

A

White to blue to red

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18
Q

Why does cyanosis occur after vasospasm in Raynaud’s?

A

As static venous blood deoxygenates

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19
Q

Why does reactive hyperaemia occur after vasospasm and cyanosis in Raynaud’s?

A

Transient increase in organ blood flow, as a response to occlusion

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20
Q

What are the clinical manifestations of SLE?

A
  • Malar Rash – Erythema that spares the nasolabial fold
  • Photosensitive rash
  • Mouth ulcers
  • Hair loss
  • Raynaud’s phenomenon
  • Arthralgia and arthritis (sometimes)
  • Serositis (pericarditis, pleuritis, less commonly – peritonitis)
  • Renal disease – glomerulonephritis (lupus nephritis)
  • Cerebral disease – ‘cerebral lupus’ – psychosis.
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21
Q

What is the pathogenesis of SLE?

A

Apoptosis leads to translocation of nuclear antigens to membrane surface

Impaired clearance of apoptotic cells results in enhanced presentation of nuclear antigens to immune cells

B-cell autoimmunity

4) Tissue damage by antibody effector mechanisms - complement activation and Fc receptor engagement

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22
Q

What risks are associated with anti-phospholipid (cardiolipin) antibodies in SLE?

A

There is an increased risk of arterial and venous thrombosis

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23
Q

What autoantibody is present in systemic vasculitis?

A

Antinuclear cytoplasmic antibodies (ANCA)

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24
Q

Which autoantibodies are screened when ANA positive?

A
  • Anti-Ro
  • Anti-La
  • Anti-centromere
  • Anti-RNP
  • Anti-ds-DNA antibodies
  • Anti-Scl-70.
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25
Q

What are the two main examples of cytoplasmic antibodies?

A

Anti-tRNA synthetase

Anti-ribosomal P antibodies

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26
Q

Which antibody is associated in neonatal lupus syndrome?

A

Anti-La

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27
Q

What is the main clinical risk in a neonate with neonatal lupus syndrome?

A

Permanent heart block - conduct ECG

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28
Q

What is the significance of anti-ribosomal P antibodies?

A

Cerebral lupus

29
Q

What inflammatory markers are raised and normal in SLE?

A

CRP is normal (Unless infection or serositis)

ESR elevated

30
Q

What haematological manifestations are found in SLE?

A

Haemolytic anaemia
Lymphopenia
Thrombocytopenia

31
Q

What renal investigations are performed in patients with SLE?

A

Measure urine protein: Creatinine ratio
Albumin
eGFR

32
Q

What immunological manifestations are found in patients with SLE?

A
  • Antinuclear antibodies
  • Anti-double-stranded DNA antibodies – highly specific, correlate with disease activity.
  • Complement consumption (Low C4 and C3) – in active disease.
33
Q

Which parameter in SLE is suggestive of active disease?

A

Complement consumption and anti-dsDNA antibodies

34
Q

Which auto-antibody is associated with thrombosis and clotting?

A

Anti-phospholipid antibodies

35
Q

How is proteinuria detected in SLE, using serum measurements?

A

Decreasing serum albumin (also a negative acute phase protein so responds to inflammation)

36
Q

Why does platelet count decrease in active SLE?

A

Due to excessive thrombotic activity

37
Q

Why does haemoglobin decrease as SLE progresses?

A

Due to autoimmune haemolytic anaemia in lupus

38
Q

What happens to the reticulocyte count in lupus?

A

Rises as a compensatory mechanism to the immune complex mediated erythrocyte destruction

39
Q

What is the recommended management for lupus?

A

Hydroxychloroquine

40
Q

What is the maintenance treatment in lupus?

A

Glucocorticoids

41
Q

What treatment is available for acute settings of lupus?

A

Glucocorticoids

42
Q

Which immunomodulatory agents are used in lupus?

A

Methotrexate, azathioprine and mycophenolate

43
Q

How should glucocorticoids be withdrawn?

A

Tapered

44
Q

What treatment is recommended in active and severe SLE disease?

A

Cyclophosphamide

B-cell targeted therapies (rituximab and belimumab)

45
Q

What is Sjogren’s syndrome?

A

Autoimmune exocrinopathy - lymphocyte infiltration of exocrine glands

46
Q

What are the exocrine related manifestations of Sjogren’s syndrome?

A

Dry eyes
Dry mouth
Parotid gland enlargement

47
Q

What is xerostomia?

A

Dry mouth

48
Q

What is xerophthalmia?

A

Dry eyes

49
Q

What are common extra-glandular manifestations of Sjogren’s syndrome?

A

Non-erosive arthritis and Raynaud’s phenomenon.

50
Q

What is secondary Sjogren’s syndrome?

A

Context of another connective tissue disorder (SLE)

51
Q

What does a salivary gland biopsy reveal in Sjogren’s glands?

A

Lymphocyte infiltration predominantly CD4 helper T-cells and to lesser extent B lymphocytes

52
Q

What is Schirmer’s test?

A

A test to assess tear production.
• Filter paper is placed under the lower lid -extent of wetness measured after 5 minutes.
• Abnormal <5mm after 5 minutes

53
Q

What is inflammatory muscle disease?

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with dermatomyositis or without polymyositis (rash).

54
Q

What skin changes occur in dermatomyositis?

A

Lilac coloured rash on eyelids (heliotrope), malar region and naso-labial

55
Q

What colour are Gottron’s papules?

A

Red-brick

• Red or purple flat or raised lesions on knuckles (Gottron’s papules).

56
Q

What are the cutaneous manifestations of dermatomyositis?

A
  • Skin changes in dermatomyositis.
  • Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds.
  • Red or purple flat or raised lesions on knuckles (Gottron’s papules).
  • Subcutaneous calcinosis
  • Mechanic’s hands (fissuring and cracking of skin over finger pads).
57
Q

Which serum markers are elevated in dermatomyositis?

A

Creatine phosphokinase

58
Q

Which cell is associated with polymyositis?

A

CD8 T-cells

59
Q

Which cell is associated with dermatomyositis?

A

CD4 T cells, in addition to B-cells

60
Q

What is systemic sclerosis?

A

Thickened skin with Raynaud’s phenomenon

Dermal fibrosis - cutaneous calcinosis and telangiectasis

61
Q

What are the manifestations of diffuse systemic sclerosis?

A
  • Fibrotic skin proximal to elbows or knees (excluding face and neck)
  • Anti-topoisomerase-I (Anti-Scl-70) antibodies.
  • Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement.
  • Short history of Raynaud’s phenomenon.
62
Q

What are the manifestations of limited systemic sclerosis?

A
  • Fibrotic skin hands, forearms, feet, neck and face
  • Anti-centromere antibodies
  • Pulmonary hypertension
  • Long history of Raynaud’s phenomenon.
63
Q

Which antibodies are associated with CREST and limited systemic sclerosis?

A

Anti-centromere antibodies

64
Q

What is CREST?

A

CREST describes a sub-type of limited systemic sclerosis.

Calcinosis, Raynaud’s phenomenon, Oesophageal dysmotility, Sclerodactyly, Telangiectasia

65
Q

What is overlap syndrome?

A
  • When features of more than 1 connective disorder are present: SLE and Inflammatory muscle disease.
  • Incomplete features of a connective tissue disease are present we can use the term undifferentiated connective tissue disease
66
Q

Which auto-antibody is associated with mixed connective tissue disorder?

A

Anti-U1 RNP antibody

67
Q

Which auto-antibody is associated with diffuse systemic sclerosis?

A

Anti-Scl-70

68
Q

What are anti-centromere autoantibodies associated with?

A

Limited systemic sclerosis

69
Q

Which autoantibodies are associated with polymyositis?

A

Anti-tRNA transferase

BRS 2 (62 decks)

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