CONNECTIVE TISSUE DISORDERS

Question 1

Name the seronegative spondyloarthropathies

Answer 1

Ankylosing spondylitis
Reactive arthritis
Psoriatic arthritis
Enteropathic synovitis

Question 2

What is systemic lupus erythmatosus (SLE)?

Answer 2

Chronic tissue inflammation in presence of auto-antibodies. Multi-site inflammation but mainly joints, skin and kidney. Typically diagnosed in women 15-45 years

Question 3

Which autoantibodies is SLE associated with?

Answer 3

Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies (causes thrombosis)

Question 4

Name some connective tissue diseases

Answer 4

SLE
Sjogren's syndrome
Autoimmune inflammatory muscle disease
Systemic sclerosis (scleroderma)
Overlap syndromes

Question 5

What symptom is common in connective tissue disorders and describe it?

Answer 5

Raynaud’s phenomenon

• Intermittent vasospasm of digits on exposure to cold
• Blanching of digit
• Cyanosis (static venous blood deoxygenates)
• Reactive hyperaemia

Colour changes of hand - white to blue to red

Question 6

How does SLE manifest?

Answer 6

Malar rash
Photosensitive rash
Mouth ulcers
Hair loss
Raynaud's phenomenon
Arthralgia/arthritis
Serositis e.g. pericarditis, pleuritis... (chest pain)
Renal disease - lupus nephritis
Cerebral disease - cerebral lupus e.g. psychosis

Question 7

What are the key auto-antibodies in RA?

Answer 7

Rheumatoid factor

Anti-cyclic citrullinated peptide antibody (ACCP/ACPA)

Question 8

What are the key auto-antibodies present in SLE?

Answer 8

Antinuclear antibodies (ANA)
Anti-double stranded DNA antibodies (anti-dsDNA)
Anti-phospholipid antibodies

Question 9

What are anti-phospholipid antibodies associated with?

Answer 9

Arterial and venous thrombosis in SLE

May also occur in absence of SLE and is termed primary anti-phospholipid antibody syndrome

Question 10

What are the key auto-antibodies present in systemic vasculitis?

Answer 10

Antinuclear cytoplasmic antibodies (ANCA)

Question 11

What investigations can you do for a patient suspected with SLE?

Answer 11

High ESR
Normal C-reactive protein unless infection/serositis/arthritis

Bloods:

• Haemolytic anaemia
• Lymphopenia
• Thrombocytopenia

ANAs
Anti-dsDNA
Antiphospholipid antibodies
Low C4/C3 (complement consumption)

Abnormal liver function, neutropenia may be present in treated patients as adverse reactions to medication

Question 12

What is a serologically active lupus patient?

Answer 12

A patient with low C3/4 and high anti-dsDNA

Question 13

Why might C3/C4 be low in a patient with SLE?

Answer 13

Autoimmune complexes activate classical pathways causing consumption of complement C3 and C4 leading to inflammation