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Systemic Lupus Erythematosus

Systemic Lupus Erythematosus. Naomi Sen. Aims and Objectives. Aim To give an outline of the diagnosis and management of SLE Objectives To describe signs and symptoms of SLE To outline relevant investigations To describe management of SLE. Outline. Pathogenesis

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Systemic Lupus Erythematosus

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  1. Systemic Lupus Erythematosus Naomi Sen

  2. Aims and Objectives • Aim • To give an outline of the diagnosis and management of SLE • Objectives • To describe signs and symptoms of SLE • To outline relevant investigations • To describe management of SLE

  3. Outline • Pathogenesis • Epidemiology and risk factors • Presentation • Investigations • Associated illnesses • Management • Pregnancy and fertility • Prognosis • Summary

  4. Systemic Lupus Erthymatosus • SLE is a heterogeneous, inflammatory, multisystem autoimmune disease in which antinuclear antibodies occur.

  5. Systemic Lupus Erthymatosus • SLE is a heterogeneous, inflammatory, multisystemautoimmune disease in which antinuclear antibodies occur. • Failure to clear apopetic material efficiently • Anti-Ro and Anti–La •  widespread vasculitis

  6. Epidemiology and risk factors • Prevalence : 50-100/100000 • Peak onset 20-40y • Female : Male 9:1 • Chinese, Southeast Asian (1 in 1000) and Afro-Caribbean (1 in 500) most common • Least common in Northern European origin (1 in 2800)

  7. Risk Factors • Genetic • HLA-B8 and DR3 in caucasians, (DR2 in Japanese) • Complement • Defective C4 gene • Environmental • EBV • UV light • Drugs: • Chlorpromazine Isoniazid • Methyldopa d-penicillamine • Hyrdalazine Minocycline

  8. Presentation (1)

  9. Presentation (2) • Relapsing and remitting • Non-specific • Fatigue • Malaise • Arthralgia • Lymphadenopathy • Fever

  10. Presentation (3) • Joints and Muscles • Most common clinical feature • Symmetrical small joint arthralgia • Clinically normal examination • Skin • Butterfly erythema • Vasculitic lesions on fingertips and nail folds • Purpura and urticaria • 1/3 - photosensitivity

  11. Presentation (4) • Lungs • Pleurisy • Recurrent pleural effusions (exudate) • Cardiovascular • Pericarditis • Mild myocarditis =/- arrhythmias • Raynaud’s • Arterial and venous thromboembolism – antiphospholipid syndrome • Atherosclerotic disease

  12. Presentation (5) • Kidneys • glomerulonephritis • CNS • Depression • Epilepsy • Migraine • Hemiplegia • Ataxia • Psychosis • Demyelinating syndromes

  13. Presentation (6) • Eyes • Retinal vasculitis  hard exudates and haemorrhages • Episcleritis/conjunctivitis/optic neuritis • GI • Mouth ulcers • Abdominal pain – mesenteric vasculitis  inflammation  perforation or infarction

  14. Diagnostic Criteria • DOPAMINE RASH – 4 out of 11 • Discoid Rash • Oral Ulcers • Photosensitivity • Arthritis • Malar rash • Immunological – anti ro, la, smith, dsDNA • Neurological changes • Elevated ESR • Renal involvement • ANA +ve • Serositis (plurisy and pericarditis) • Haematological (haemolytic anaemia, ↓WCC↓plt)

  15. Investigations • Bloods • FBC • Leucopenia/ Lymphopenia • Thrombocytopenia • Anameia –AI haemolysis • ESR • Raised, CRP normal • ANA • Positive • RF +ve – 25% • Complement levels • Reduced • Antiphospholipid antibodies • Anticardiolipn • Anti B2-glycoprotein • Lupus anticoagulant • Immunoglobulins • Raised • Polyclonal • IgG and IgM

  16. Investigations • Histology • Skin biopsies • Renal biopsies • Imaging • CT • MRI

  17. Associated diseases • Antiphospholipidsyndrome • Overlap syndromes: scleroderma, polymyositis, rheumatoid arthritis and Sjögren'ssyndrome • Prone to other autoimmune conditions such as thyroiditis • Higher incidence of drug allergy • Increased risk of infection • Increased risk atherosclerosis, hypertension, dyslipidaemias, diabetes, osteoporosis, avascular necrosis and malignancies (especially non-Hodgkin's lymphoma)

  18. Management • Individual counselling • Avoid sun exposure , use sunscreen • Analgesia – caution with NSAIDs • Corticosteroids – effecitive – but s/e • Hydroxychloroquine • Cyclphosphamide – life threatening • Azothioprine – steroid sparing • Methotrexate

  19. Contraception • Barrier methods of contraception -safest • Oestrogens can exacerbate lupus • Lowest dose COCP can be used with caution if no • Migraines • Thrombosis • Hypertension • Anticardiolipin antibodies are negative • Increased risk of thrombosis – needs to be counselled.

  20. Pregnancy and fertility • Fertility is normal • Pregnancy is safe in mild or stable disease • In severe lupus – disease should be controlled prior to pregnancy • Morbidity - ↑if antiphospholipid antibodies • Recurrent miscarriage • Pre-eclampsia • IUGR • Premature delivery • Thrombosis • Worsening or renal disease and hypertension • LMWH and low dose aspirin treatment of choice

  21. Prognosis • Improved with earlier recognition and improved management. • Morbidity and mortality – higher in patients with extensive multisystem disease and multiple auto antibodies • Renal involvement  poorer prognosis • Drug induced lupus usually subsides when the drug is discontinued.

  22. Summary (1) • SLE is a heterogeneous, inflammatory, multisystemautoimmunedisease in which antinuclear antibodies occur. • More common in females in their 20-40s • More common in Asian and Afro-Caribbean populations

  23. Summary (2) • Relapsing remitting • Diagnosis – 4 out of 11 “DOPAMINE RASH” • Multiple systems – most common – joint pain • Initial investigations – Bloods - FBC, ESR, CRP, Complement, ANA, RF, Immunoglobulins, Antiphospholipid antibodies

  24. Summary (3) • Management • Individual • Sunscreen • Analgesia • Steroids and immunosuppression if severe • Contraception if severe • Barrier – fewer risks than hormonal • If antiphospholipid • LMWH and aspirin in pregnancy

  25. Questions

  26. References • www.patient.co.uk • Kumar and Clarke • Also Wikipedia and Dr Google!

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