Sle Dev

Systemic lupus
Erythematosus(SLE)
Introduction
 SLE is an chronic inflammatory multisystem autoimmune disease that can

affect the skin, joints, kidneys, lungs, nervous system, serous membranes, and/or
other organs of the body.
 Immunologic abnormalities, especially the production of a number of
antinuclear antibodies, are prominent feature of the disease.
 The clinical course of SLE is variable and may be characterized by periods of
remissions and of chronic or acute relapses.
 Around 90% of are F, the peak age at onset is between 20-30 yrs.
 SLE is the classical example of systemic autoimmune or
Collagen diseases. The disease derives its name ‘lupus’
from the Latin word meaning ‘wolf’ since initially this
disease was believed to affect skin only and eat away skin
like a wolf.
Natural history and course
2 Forms of Lupus Erythematosus:-
 1. Systemic or disseminated form is characterised by Acute

and chronic inflammatory lesions widely scattered in the body and
there is presence of various nuclear and cytoplasmic autoantibodies
in the plasma.
 2. Discoid form is characterised by chronic and localised skin
lesions involving the bridge of nose and adjacent cheeks without
any systemic manifestations. Rarely, discoid form may develop into
disseminated form.
Etiology
 The exact etiology of SLE is not known. However, there is role of heredity and
certain environmental factors:
 2. Genetic factors. Genetic predisposition to develop auto-antibodies to
nuclear and cytoplasmic antigens in SLE is due to the immunoregulatory
function of class II HLA genes implicated in the pathogenesis of SLE.
 3. Environmental factors. Various other factors express the genetic
susceptibility of an individual to develop clinical disease. These factors are:
 i) certain drugs e.g. Penicillamine D;
 ii) certain viral infections e.g. EBV infection; and
 iii) certain hormones e.g. oestrogen
Pathogenesis
 Interaction between susceptibility genes and environmental factors results in abnormal

Immune responses by formation of various autoantibodies. Autoantibodies against nuclear
and cytoplasmic components of the cells are demonstrable in plasma by immunofluorescence
tests in almost all cases of SLE. Some of the important antinuclear antibodies (ANAs) or
antinuclear factors (ANFs) Against different nuclear antigens are as under:
 1. Antinuclear antibodies (ANA)- Demonstrated in about 98% cases and are used as
Screening test.
 2. Antibodies to Double-stranded (Anti-dsDNA)- most specific for SLE , present in 70% of
cases
 3.Anti-Smith antibodies (anti-Sm) – Seen in 25% Cases .
 4. Other non specific antibodies
LE CELL PHENOMENON
 This was the first diagnostic laboratory test

described for SLE.
 The binding of exposed nucleus with ANAs
results in homogeneous mass of nuclear
chromatin material which is called LE body
or Haematoxylin body.
 LE cell test positive in 70% cases of SLE.
Morphological features
Morphological features The manifestations of SLE are widespread in different

visceral organs and as erythematous cutaneous eruptions. The principal lesions are
renal, vascular, cutaneous and cardiac; other organs and tissues involved are serosal
linings (pleuritis, pericarditis), joints (synovitis), spleen (vasculitis), liver (portal
triaditis), lungs (interstitial pneumonitis, fibrosing alveolitis), CNS (vasculitis) and
in blood (autoimmune haemolytic anaemia, thrombocytopaenia).
 Histologically, the characteristic lesion in SLE is fibrinoid necrosis which may
be seen in the connective tissue, beneath the endothelium in small blood vessels,
under the mesothelial lining of pleura and pericardium, under the Endothelium in
endocardium, or under the synovial lining cells of joints.
Clinical Features
SLE, like most other auto immune diseases, is more common in women in their 2 nd
to 3rd decades of life. SLE is a multisystem disease and thus a wide variety of
clinical features may be present. The severity of disease varies from mild to
intermittent to severe and fulminant. Usually targeted organs are musculoskeletal
system, skin, kidneys, nervous system, lungs, heart and blood vessels, GI system,
and haematopoietic system. Fatigue and myalgia are present in most cases
throughout the course of disease. Severe form of illness occurs with fever, weight
loss, anaemia and organ related manifestations.
 The disease usually runs a long course of fl are-ups and remissions; renal failure
is the most frequent cause of death.

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Systemic lupus

 SLE is an chronic inflammatory multisystem autoimmune disease that can

 1. Systemic or disseminated form is characterised by Acute

 Interaction between susceptibility genes and environmental factors results in abnormal

 This was the first diagnostic laboratory test

Morphological features The manifestations of SLE are widespread in different

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