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Systemic Lupus Erythematosus
Systemic Lupus Erythematosus (SLE)

Multisystemic inflammatory chronic disease
characterized by inflammation of blood vessels and
connective tissues, caused by presence of activated
helper T – cells that induce a B cell response, resulting
in the secretion of pathogenic autoantibodies and
formation of immune complexes.
OR

SLE is an autoimmune disease, which means that the
body's natural defense system (immune system)
attacks its own tissues instead of attacking foreign
substances like bacteria and viruses.
Etiology:

Cause unknown
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Many factors play a role in immune dysregulation
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Genetic (HLA types B8, DR2, DR3)
Hormones (attacks during menses, pregnancy due to
Estrogen )
Environmental factors (ultra voilet ray viruses e.g; EBV )
Drugs induced lupus (anticonvulsants, salfonamides,
penicillamine)
Non specific response to an antigenic stimulus/ loss of
tolerance to self antigen

Polyclonal activation of B cells

 Immunoglobulins & autoantibodies
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Form circulating and tissue bound immune complexes
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Complement fixation & recruitment (screening) of inflammatory cells
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TISSUE INJURY
•Fibrinoid deposits found on
blood vessel walls and
affected organs.
Rheumatoid nodules
and granulomas maybe
present

Auto antibodies are formed against DNA & other nuclear
antigens, ribosomes, platelets, coagulation factors,
immunoglobulins, erythrocytes, leukocytes
Epidemiology

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Incidence unknown
Vary depending on ethnicity and location 4250/100,000 population
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Age: Unusual before 8 yrs (have been
diagnosed even in first yr of life)

Sex: Female predominance 1:4 before
puberty to 1:8 after puberty
Diagnosis

Criteria for diagnosis require presence
of 4 of 11 criteria serially or
simultaneously , during any interval of
observation.

American College of Rheumatology Classification Criteria for
Systemic Lupus Erythematosus

1. Malar rash: fixed erythema, flat or raised, over the malar
eminences, tending to spare the nasolabial folds
Discoid rash

2. Discoid rash: erythematous, raised patches with adherent
keratotic scaling and follicular plugging; possibly atrophic
scarring in older lesions
Cont …
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3. Photosensitivity: skin rash as a result of
unusual reaction to sunlight, as determined by
patient history or physician observation

4. Oral ulcers: oral or nasopharyngeal
ulceration, usually painless, observed by
physician

5. Arthritis: non – erosive arthritis involving
two or more peripheral joints, characterized
by swelling, tenderness, or effusion

6. Serositis: - pleuritis, by convincing history of pleuritic
pain, rub heard by physician, or evidence of pleural
effusion; or
--- pericarditis documented by
electrocardiography, rub heard by physician, or evidence of
pericardial effusion

7. Renal disorder:---- persistent proteinuria, > 500 mg per
24 hours (0.5 g per day) or > 3+ if quantitation is not
performed;
or
---- cellular casts (may be red blood cell,
hemoglobin, granular, tubular, or mixed cellular casts)

8. Neurologic disorder: ---- seizures or psychosis occurring
in the absence of offending drugs or known metabolic
derangement (e.g., uremia, ketoacidosis, electrolyte
imbalance)
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9. Hematologic disorder:
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hemolytic anemia with reticulocytosis; or
leukopenia, < 4,000 per mm3 on two or more
occasions; or
lymphopenia, < 1,500 per mm3 on two or more
occasions; or
thrombocytopenia, < 100x103 per mm3 in the
absence of offending drugs
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10. Immunologic disorder: antibody to double-stranded
DNA antigen (anti-dsDNA) in abnormal titer; or
presence of antibody to Sm nuclear antigen (antiSmith); or positive finding of antiphospholipid
antibody based on :1. an abnormal serum level of IgG or IgM anticardiolipin
antibodies,
2. a positive test result for lupus anticoagulant using a
standard method, or
3. a false-positive serologic test for syphilis that is known
to be positive for at least 6 months and is confirmed by
negative Treponema pallidum immobilization or
fluorescent treponemal antibody absorption test
11. Antinuclear antibodies: an abnormal antinuclear antibody titer by
immunofluorescence or equivalent assay at any time and in
the absence of drugs known to be associated with drug-induced
lupus
Serology
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Almost all patient with SLE have demonstrable
ANAs
Anti – double – stranded DNA antibodies is highly
specific
Anti – histone antibodies (drug induced lupus
erythematous)
Anti – Sm antibodies (CNS lupus)
Treatment

Depend on affected target organ and disease
severity
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Glucocorticoids form the mainstay of therapy.
Prednisolone is usually started in doses of 1 – 2
mg/kg/day and then gradually tapered according to
disease activity.
NSAIDs for arthralgia and arthritis
Sunscreen lotions 3 – 4 times/days.
Cont…
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Life – threatening complications (nephritis,
myocarditis, encephalopathy):
------ use of I.V of methyl prednisolone or
dexamethasone for 3 – 5 days, followed by oral
prednisolone.
Severe lupus nephritis ------ I.V cyclophosphamide
IVIG (nephritis, vasculitis and acute
thrombocytopenia)
Antibiotics
Cont…
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For almost all patients with SLE, low dose prednisolone
(2.5 – 5 mg/kg) has to be continued for many years.
After stable, hydroxychloroquine (5 – 6 mg/ kg): for mild
manifestations (skin lesions, arthritis arthralgia) and
further tapering of steroids.

Side effects of Steroids include :

fluid retention, weight gain, high blood pressure, potassium
loss, headache, muscle weakness, puffiness of and hair growth
on the face, thinning and easy bruising of the skin, glaucoma,
cataracts, peptic ulceration, worsening of diabetes, irregular
menses, growth retardation in children, convulsions, and
psychic disturbances (depression, euphoria, insomnia, mood
swings, personality changes, and even psychotic behavior).
Cont…
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Minor manifestations respond to NSAIDs, low dose
corticosteroids, hydroxychloroquine or methotrexate
Major manifestations (immunosuppressive therapy) --- high dose of corticosteroids, azathioprine,
methotrexate, cyclosporin and cyclophospamide
Mycophenolate mofetil (new drugs) those not
responding to cyclophosphamide or azathioprine.

Anticoagulant medications: Thrombosis,
antiphospholipid antibodies or lupus
anticoagulant
Prognosis

Childhood SLE : 5 yr survival 90%

Major cause of death include
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Infection
CNS complications
Pulmonary hemorrhage
Myocardial infarction