1. Naomi Sen

2.  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.  Pathogenesis  Epidemiology and risk factors  Presentation  Investigations  Associated illnesses  Management  Pregnancy and fertility  Prognosis  Summary

4.  SLE is a heterogeneous, inflammatory, multisystem autoimmune disease in which antinuclear antibodies occur.

5.  Failure to clear apopetic material efficiently  Anti-Ro and Anti–La   widespread vasculitis

7.  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.  Genetic ◦ HLA-B8 and DR3 in caucasians, (DR2 in Japanese)  Complement ◦ Defective C4 gene  Environmental ◦ EBV ◦ UV light ◦ Drugs:  ChlorpromazineIsoniazid  Methyldopad-penicillamine  HyrdalazineMinocycline

10.  Relapsing and remitting  Non-specific ◦ Fatigue ◦ Malaise ◦ Arthralgia ◦ Lymphadenopathy ◦ Fever

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

13.  Kidneys ◦ glomerulonephritis  CNS ◦ Depression ◦ Epilepsy ◦ Migraine ◦ Hemiplegia ◦ Ataxia ◦ Psychosis ◦ Demyelinating syndromes

14.  Eyes ◦ Retinal vasculitis  hard exudates and haemorrhages ◦ Episcleritis/conjunctivitis/optic neuritis  GI ◦ Mouth ulcers ◦ Abdominal pain – mesenteric vasculitis  inflammation  perforation or infarction

15.  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.  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.  Histology ◦ Skin biopsies ◦ Renal biopsies  Imaging ◦ CT ◦ MRI

18.  Antiphospholipid syndrome  Overlap syndromes: scleroderma, polymyositis, rheumatoid arthritis and Sjögren's syndrome  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.  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.  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.  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.  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.  SLE is a heterogeneous, inflammatory, multisystem autoimmune disease in which antinuclear antibodies occur.  More common in females in their 20-40s  More common in Asian and Afro-Caribbean populations

24.  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.  Management ◦ Individual ◦ Sunscreen ◦ Analgesia ◦ Steroids and immunosuppression if severe ◦ Contraception if severe  Barrier – fewer risks than hormonal ◦ If antiphospholipid  LMWH and aspirin in pregnancy

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