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Systemic Lupus Erythematous (Signs (Raynaud's (Initial pallor…
Systemic Lupus Erythematous
Definition
SLE is a multi-system autoimmune disease where antibodies are made against a variety of autoantigens (ANA) which form immune complexes
SLE is a connective tissue disease
Epidemiology
Prevalence is 0.2%
Highest incidence in Afro-Caribbeans
F:M = 9:1
Aetiology
Higher incidence in premenopausal women - oestrogen has an effect
Drugs can cause SLE e.g. hydralazine, procainamide
Associated with HLA-B8/DR2/DR3
UV light triggers flares
Hereditary condition
EBV
Pathogenesis
Self antigens are presented to T cells
They stimulate B cells to produce autoantibodies against the nuclear antigens
These are transferred to lymphoid tissue
Antibody formation, immune complex deposition, complement activation + influx of neutrophils = clinical manifestations
Apoptopic cells and fragments aren't cleared efficiently by phagocytes
95% of patients are ANA +ve
Signs
Serositis *
Non scarring alopecia*
Oral ulcers*
Photosensitive rash*
Synovitis*
Butterfly rash on face *
Proteinuria*
Leucopenia + thrombocytopenia*
Haemolytic anaemia*
Raynaud's
Initial pallor (vasoconstriction) followed by cyanosis, and then redness from hyperaemia
Raynaud's disease = Raynaud's phenomenon but with no underlying disorder
Precipitated by cold and relieved by heat
Treat by keeping hands and feet warm, stop smoking and stop beta blockers
This is intermittent spasms in the arteries supplying the fingers and toes
Lymphadenopathy
Neurological *
Anxiety
Seizures
Cranial neuropathy
Psychosis
Myasthenia gravis
Investigations
ESR is high but CRP is normal
Complement: low C3/C4
Double stranded DNA antibody is specific to SLE
Skin or renal biopsy
WHO criteria: 4+ of 11 criteria (signs *)
ANA +ve
Treatment
Give topical steroids + sunscreen to cuteneous symptoms
Maintenance
Hydroxychloroquine
Low dose steroids
NSAIDs
Methotrexate
Give high dose prednisolone in severe flares
Lupus Nephritis
Cyclophosphamide
More intense immunosuppression
UV protection
Prognosis: 80 survival at 15 years, increased long term risk of CVD and osteoporosis