Systemic Lupus Erythematosus

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

7. 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)

8. 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

9. Presentation (1)

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

11. 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

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

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

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

15. 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)

16. 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

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

18. 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)

19. 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

20. 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.

21. 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

22. 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.

23. 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

24. 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

25. 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

26. Questions

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