Systemic Lupus Erythematosus

Question 1

What is lupus?

Answer 1

Systemic autoimmune inflammatory disease
Multi-systems pathology
Females 10x more likely, probably young when started

Question 2

Pathogenesis?

Answer 2

1. Genetic factors (HLA-DR3) + environmental factors (UV exposure, microbial response)
2. Abnormal clearance of apoptotic cell material
3. Dendritic cell uptake of autoantigens
4. Causes hyperreactive B-cell activation -> autoantibody production
5. Leading to self-recognition and apoptosis

Question 3

Clinical features?

Answer 3

SOAP BRAIN MD

Serositis (inflammation of serous tissues)
Oral ulcers
Arthritis
Photosensitivity

Blood (ALL low)
Renal proteinuria
Immunological (ANA, anti-dsDNA)
Neurological seizures/psychosis

Malar rash
Discoid rash

(ALSO Reynaud’s phenomenon - fingers go white/blue/red due to spasm of arteries)

Question 4

Differential Diagnosis

Answer 4

ANA positive (but occurs in other autoimmune diseases)
Other autoimmune connective tissue disease
Drug induced Lupus

Question 5

Diagnosis?

Answer 5

ANA antibodies (flourescently marked, non-specific)
HOMOEGENOUS ANA = Lupus

Anti-dsDNA (more specific but less sensitive)

Question 6

How do you assess disease severity?

Answer 6

1. Identify pattern of organ involvement
2. Monitor function of affected organs
3. Pattern of autoantibodies expressed (anti-dsDNA)

Question 7

How do you prevent/predict severe attacks?

Answer 7

Clinical features:
Weight loss, fatigue, malaise, hair loss, rash

Lab markers:
Increased anti-dsDNA

Question 8

Treatment?

Answer 8

Mild disease (joint + skin)

• Paracetamol/NSAID (monitor renal function)
• Topical corticosteroids
• Hydroxychloroquine

Moderate/Severe disease (Inflammation in non-vital/vital organs)

• Oral steroids or IV corticosteroid (methyprednisolone)
• Immunosuppressants (cyclophosphamide) if multiple organs involved
• Maintenance treatment = low dose oral steroids + immunosuppressants