Systemic Lupus Erythematous

Question 1

Define:

Answer 1

• Multi-system inflammatory autoimmune disorder in which autoantibodies are made against a variety of autoantigens

Question 2

What is the diagnostic criteria:

Answer 2

4/11 of SOAP BRAIN MD:
o Serositis – pleuritis or pericarditis
o Oral ulcers
o Arthritis (non-erosive)
o Photosensitivity
o Bloods (haemolytic anaemia/leukopaenia/thrombocytopaenia)
o Renal disease (urine casts/proteinuria)
o ANA
o Immunological disorder (anti-dsDNA/anti-Sm/anti-phospholipid)
o Neurological disease (psychosis/seizures)
o Malar rash
o Discoid rash

Question 3

Aetiology:

Answer 3

• UNKNOWN
• Tissue damage may be caused by vascular immune complex deposition
• Could be due to a combination of hormonal, genetic and exogenous factors
• Association with HLAB8, DR2 and DR3

Associated with other autoimmune diseases such as hyperthyroidism and Sjogrens

Question 4

Epidemiology:

Answer 4

• COMMON
• 1-2/1000
• More common in the YOUNG
• More common in AFRO-CARIBBEAN and CHINESE
• 9 x more common in FEMALES

Question 5

Signs and symptoms:

Answer 5

•	RELAPSING AND REMITTING
•	SOAP BRAIN MD
•	General Symptoms
o	Fever 
o	Fatigue 
o	Weight loss 
o	Lymphadenopathy
o	Splenomegaly
•	Raynaud's phenomenon – numbness of fingers/toes in cold
•	Oral ulcers 
•	Skin Rash
o	Malar rash 
•	Discoid lupus (red scaly patches)
•	Atypical rashes (e.g. photosensitivity, vasculitis, urticaria, purpura)

Question 6

Investigations:

Answer 6

•	Bloods
o	FBC
o	U&E
o	LFT 
o	Raised ESR 
o	Normal CRP
o	Clotting 
o	Complement – consumption of complement causes low C3 and C4 but high C3d and C4d (degradation products)
•	Autoantibodies
o	Anti-dsDNA (60%)
o	Rheumatoid factor (30-50%)

• Urine - haematuria, proteinuria, red cell casts
• Joints - plain radiographs
• Heart and Lungs - CXR, ECG, echocardiogram, CT
• Kidneys - renal biopsy (if glomerulonephritis suspected)
• CNS - MRI scan, lumbar puncture