Systemic Lupus Erythematosus

Naomi Sen

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

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

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

SLE is a heterogeneous,

inflammatory, multisystem

autoimmune disease in which

antinuclear antibodies occur. Failure to clear apopetic material efficiently Anti-Ro and Anti–La widespread vasculitis

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)

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

Relapsing and remitting Non-specific

◦ Fatigue◦ Malaise◦ Arthralgia◦ Lymphadenopathy◦ Fever

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

Lungs ◦ Pleurisy◦ Recurrent pleural effusions (exudate)

Cardiovascular◦ Pericarditis◦ Mild myocarditis =/- arrhythmias◦ Raynaud’s◦ Arterial and venous thromboembolism –

antiphospholipid syndrome◦ Atherosclerotic disease

Kidneys◦ glomerulonephritis

CNS◦ Depression◦ Epilepsy◦ Migraine◦ Hemiplegia◦ Ataxia◦ Psychosis◦ Demyelinating syndromes

Eyes◦ Retinal vasculitis hard exudates and

haemorrhages◦ Episcleritis/conjunctivitis/optic neuritis

GI◦ Mouth ulcers◦ Abdominal pain – mesenteric vasculitis

inflammation perforation or infarction

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)

Histology◦ Skin biopsies◦ Renal biopsies

Imaging ◦ CT◦ MRI

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)

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

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.

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

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.

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

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

Management◦ Individual◦ Sunscreen◦ Analgesia◦ Steroids and immunosuppression if severe◦ Contraception if severe

Barrier – fewer risks than hormonal◦ If antiphospholipid

LMWH and aspirin in pregnancy

www.patient.co.uk Kumar and Clarke

Also Wikipedia and Dr Google!