SLE

Question 1

What is SLE?

Answer 1

Inflammatory autoimmune connective tissue disease

Systemic - can affect multiple organs
Erythematous - malar rash

Question 2

Course of SLE

Answer 2

Relapsing-remitting

Chronic inflammation means patients with lupus often have shortened life expectancy

Question 3

Leading cause of death in SLE

Answer 3

Cardiovascular disease

Infection

Question 4

Antibody associated with SLE

Answer 4

Anti-nuclear antibodies

85% of patients with SLE will be positive for ANA

Question 5

Pathophys of SLE

Answer 5

Antibodies attack proteins in cell nucleus

Immune system to target these proteins
When activated, immune system generates inflammatory response

Question 6

Presentation of SLE (12)

Answer 6

Fatigue

Weight loss

Fever

Hair loss

Photosensitive malar rash. This is a “butterfly” shaped rash across the nose and cheek bones that gets worse with sunlight

Mouth ulcers

Shortness of breath

Pleuritic chest pain

Lymphadenopathy and splenomegaly

Myalgia (muscle pain)

Arthralgia (joint pain) and non-erosive arthritis

Raynaud’s phenomenon

Question 7

Investigating SLE

Answer 7

Autoantibodies

Full blood count (normocytic anaemia of chronic disease)

C3 and C4 levels (decreased in active disease)

CRP and ESR (raised with active inflammation)

Immunoglobulins (raised due to activation of B cells with inflammation)

Urinalysis and urine protein:creatinine ratio for proteinuria in lupus nephritis

Renal biopsy can be used to investigate for lupus nephritis

Question 8

What autoantibodies are specific to SLE?

Answer 8

Anti-double stranded DNA (anti-dsDNA)

70% of patients with SLE will have anti-dsDNA antibodies

Question 9

All types of antinuclear antibody

Answer 9

Anti-Smith (highly specific to SLE but not very sensitive)

Anti-Ro and Anti-La (most associated with Sjogren’s syndrome)

Anti-centromere antibodies (most associated with limited cutaneous systemic sclerosis)

Anti-Scl-70 (most associated with systemic sclerosis)

Anti-Jo-1 (most associated with dermatomyositis)

Question 10

antiphospholipid syndrome and SLE

Answer 10

Occur in up to 40% of patients with SLE

Associated with an increased risk of venous thromboembolism

Question 11

Diagnosis of SLE

Answer 11

SLICC Criteria

Presence of antinuclear antibodies

Establishing a certain number of clinical features suggestive of SLE

Question 12

Complications of SLE

Answer 12

Infection

Anaemia of chronic disease

Cardiovascular disease - leading cause of death

Pericarditis

Pleuritis

Interstitial lung disease

Lupus nephritis

Neuropsychiatric SLE - optic neuritis, transverse myelitis, psychosis.

Recurrent miscarriage

Question 13

First line treatments of SLE

Answer 13

NSAIDs

Steroids (prednisolone)

Hydroxychloroquine (first line for mild SLE)

Suncream and sun avoidance for the photosensitive the malar rash

Question 14

Other commonly used immunosuppressants in resistant or more severe lupus

Answer 14

Methotrexate
Leflunomide

Mycophenolate mofetil
Tacrolimus
Ciclosporin
Azathioprine

Question 15

Biological therapies

Answer 15

Where patients have not responded to other treatment

Rituximab
Belimumab