Overview

• Multisystem autoimmune disease that predominantly affects women of childbearing age
• Type 3 hypersensitivity reaction
• F>M (9:1), onset usually at 20-40 yrs old, more common in Afro-Caribbeans
• Aetiology → genetic predisposition (HLA-DR2 and HLA-DR3), hormonal factors (hyperoestrogenic states), environmental factors (cigarette smoking)
• Associated with Anti-Phospholipid Syndrome → increased risk of thrombus formation (recurrent PEs and DVTs) and recurrent miscarriages. Prolonged APTT (paradoxical) and low platelets (thrombocytopenia). Associated with anti-cardiolipin antibodies. Managed with low dose aspirin (no previous thromboembolic event) or lifelong warfarin (after inital thromboembolic event - avoid warfarin in pregnant women).
  • CLOT = clots (thrombosis), liverdo reticularis, obstetric complications (miscarriages), thrombocytopenia
• Amboss Overview

Making Diagnosis

Clinical Features:

• Malar (Butterfly) Rash → erythema over the cheeks and bridge of the nose, sparing the nasolabial folds
• Photosensitive Rash → rash occurs after sun exposure. Can be painful and pruritic and usually lasts a few days, healing without scarring
• Joints → arthritis and arthralgia. Distal symmetrical polyarthritis.
• Oral Ulcers
• Constitutional Symptoms → fatigue, fever, weight loss
• Raynaud’s Phenomenon → colour changes of the digits induced by cold (white → blue → red)
• CVS → pericarditis (and pleural effusion) or myocarditis