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Pathology

Chronic inflammatory multisystem disease that follows a relapsing and remitting course, and characterized by autoantibody response to nuclear and cytoplasmic antigens

Aetiology

Unknown aetiology.
Peak in Afro-Caribbean women 25-35 years
Associated with epilepsy, digital infarcts, alopecia and pleurisy

Signs

Malar ‘butterfly’ rash, discoid rash, episcleritis, optic neuritis, Raynaud’s phenomenon, Sjögren’s syndrome, mucosal ulceration, symmetrical arthritis

Symptoms

Arthralgia, fever, malaise

Investigations

Bloods: FBC shows pancytopenia, Raised ESR & CRP, Decreased C3/C4 Levels
Auto-Antibodies: ANA, Anti-dsDNA, Anti-Sm (Most specific antibody for SLE), 
anti-cardiolipin, anti-Ro/La, anti-histone.
Renal Biopsy: Lupus nephritis.

Treatment

Medical: NSAIDs for arthritis, corticosteroids, immunosuppression

Prognosis

80% survival at 15 years. 
Increased long term risk of cardiovascular disease and osteoporosis 
Cardiovascular: Pericarditis, myocarditis, endocarditis (Libman-Sacks)
Neurological: Stroke, fits, psychosis
Renal: 50% develop lupus nephritis 
Respiratory: ARDS, pleural effusion

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Key References

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