Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Erythematosus (SLE)
• SLE is the classic model of autoimmune disease
• SLE is a systemic rheumatic disorder and the term
used most often for the group of disorders that
includes SLE and other multi system disorders
• Lupus erythematosus = "red wolf" (erythematous
rash)
• There are 11 criteria for the diagnosis of SLE and for a
Overview definitive diagnosis, patients must meet at least FOUR
of these criteria
• Two of the criteria are a positive ANA and the
detection of antibodies to Sm, dsDNA or cardiolipin
• 20-30% of SLE patient have antibodies to
Sm (high probability of SLE)
• 60% of SLE patients have antibodies to
dsDNA (indicator of ACTIVE SLE)
• 10-15 times more common in FEMALES
• More common in BLACKS than in whites
• Around 1.4 million Americans have lupus
• Survival rate is estimated to be higher than 90%
EPIDEMIOLOGY at 10 years after diagnosis
• Highest mortality rate: patients with renal or
CNS involvement
• Two most frequent cause of death: RENAL
FAILURE and infectious complications
• Idiopathic
• Primary defect in the regulation of the
immune system is considered important in
the pathogenesis of the disorder
• Genetic
• Hormonal
• estrogen, thus more common in
ETIOLOGY FEMALES
• Antiphospholipid syndrome:
associated with miscarriages
• Environmental factors
• UV light, bacterial and viral infections
• Alteration in the DNA secondary to UV
exposure stimulates autoantibody
production esp. anti-DNA
• Fever >37.8°C
• Weight loss
• Malaise
• Musculoskeletal
• Arthralgia, arthritis, osteonecrosis
• Cutaneous: "Butterfly rash" over the bridge of
Antibodies (ANAs) associated with SLE skin disease and neonatal SLE
syndrome
4. Anti-Lane(La) soluble substance-B (SS-B) -
associated with primary and secondary Sjogren's
syndrome, presence in SLE indicates MILD disease.
Presence ALONE indicates primary Sjogren syndrome
5. Membrane specific antibodies to neutrophils and
platelets and autoantibodies to lymphocytes:
SPECIFIC to SLE
IV. Antibody to nucleolar antigens
Patterns 3. Speckled
• GRAINY pattern with numerous ROUND DOTS
of nuclear fluorescence, without staining of
the nuclei
• Occurs in the presence of antibody to
extractable nuclear antigen devoid of DNA or
histone (anti-RNP, anti-Sm)
4. Nucleolar
• Reflects an antibody to nucleolar RNA (4-6s
RNP)
• Present in about 50% of patients with
Indirect scleroderma (PSS) and Sjogren's syndrome
and SLE
Immunofluorescence 5. Centromeric (discrete, speckled)
• Anti-centromere antibody reacts with
Patterns centromeric chromatin of metaphase and
interphase cells
• CREST syndrome (Calcinosis, Raynaud's
phenomenon, Esophageal dysfunction,
Sclerodactily, Telangiectasia)
DIFFUSE/
HOMOGENOUS
PERIPHERAL RIM
SPECKLED
NUCLEOLAR
CENTROMERE
Rapid Slide Test for • SLE latex test provides a suspension of
polystyrene latex particles coated with DNP