Transcript 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.




heterogeneous,
inflammatory, multisystem
autoimmune disease in which
antinuclear antibodies occur.
SLE is a
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
 Methyldopa
 Hyrdalazine
Isoniazid
d-penicillamine
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)

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

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 nonHodgkin'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.



heterogeneous,
inflammatory, multisystem
autoimmune disease in which
antinuclear antibodies occur.
SLE is a
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!
