Systemic Lupus Erythematosus

Systemic Lupus Erythematosus

(or SLE for short)

Name(s)_____________________
_
• Systemic Lupus Erythematosus
• Abbreviations: SLS or Lupus
• Alternative Names: Disseminated lupus
erythematosus; erythematosus; Discoid lupus

Find out more about Asthma Treatment Systemic Lupus Erythematosus

Definition____________________
A chronic inflammatory condition caused by
an autoimmune disease. An autoimmune
disease occurs when the body's tissues are
attacked by its own immune system. Patients
with lupus have unusual antibodies in their
blood that are targeted against their own
body tissues.


Aetiology_____________________
• No definitive cause

BUT…


Aetiology ____________________
Inate Susceptibility Environmental Stimuli
•HLA type •UV exposure
•Immunoregulatory genes •Microbial response
•Hormonal levels •Medication
•Complement levels

Autoantibody
Autoimmune Proliferation Production
•Hyperactive B-cell/T-cell activation •Apoptosis and self
•High ratio of CDA;CD8 T-cells exposure
•Defective immune complex clearance •Self-recognition
•Impaired tolerance •Foreign-Ab cross reaction


Epidemiology____________________
• Prevalence of SLE in the population:
20 to 150 cases per 100,000
• In women, prevalence rates vary from:
164 (Caucasian) per 100,000
to 406 (African American) per 100,000


Epidemiology____________________
• Due to improved detection of mild disease,
the incidence nearly tripled in the last 40
years of the 20th century.
• Estimated incidence rates are 1 to 25 per
100,000 in North America, South America,
Europe and Asia.


Epidemiology____________________
Geographic and racial distribution
• More common in urban than rural areas
• Prevalence of SLE is higher among: Asians, Afro-
Americans, Afro-Caribbeans, and Hispanic Americans
compared with Americans of European decent in the
United States and among Asian Indians compared with
Caucasians in Great Britain. In comparison, SLE occurs
infrequently in Blacks in Africa.
• In New Zealand, the prevalence and mortality of SLE are
higher in Polynesians than in Caucasians.


Epidemiology____________________
Geographic and racial distribution (symptoms)
• Photosensitivity and discoid skin lesions may be
more frequent clinical manifestations in patients
with Northern European than those with Southern
European ancestry; the former group is, however,
less likely to have anti-cardiolipin and anti-dsDNA
antibodies.


Epidemiology____________________
Gender
• The increased frequency of SLE among women has
been attributed in part to an estrogen hormonal
effect.
• In children, in whom sex hormonal effects are
presumably minimal, the female-to-male ratio is 3:1.
• In adults, especially in women of child-bearing years,
the ratio ranges from 7:1 to 15:1.
• In "older" individuals, especially post-menopausal
women, the ratio is approximately 8:1.


Epidemiology____________________
Onset
• Onset of SLE is usually after puberty, in 20s and 30s.
• 20% of cases diagnosed during first 2 decades of life.
• Prevalence is highest among women 14-64 years old.
• SLE does not have an age predilection in males,
although it should be noted that among older adults,
the female-to-male ratio falls


Pathogenesis__________________
• Autoimmune disorder
• Characterized by multisystem microvascular
inflammation with the generation of autoantibodies.
• Although the specific cause of SLE is unknown,
multiple factors are associated with the
development of the disease, including genetic, racial,
hormonal, and environmental factors


Presenting Features____________
• fever, rash, arthritis, alopecia, and renal
involvement
Symptoms Occurrence
Achy joints (arthralgia) 95%
Fever more than 38 degrees C 90%
Rheumatoid arthritis 90%
Prolonged or extreme fatigue 81%
Skin rashes 74%
Anaemia 71%
Kindey involvement 50%
Pain in the chest on deep breathing (pleurisy) 45%


Arthritis (Swollen Joints)


• fever, rash, arthritis, alopecia, and renal
involvement
Symptoms Occurrence
Butterfly-shaped rash across the cheeks and nose 42%
Sun or light sensitivity (photosensitivity) 30%
Hair loss 27%
Abnormal blood-clotting problems 20%
Raynaud’s phenomenon (fingers turning white 17%
and/or blue in the cold)
Seizures 15%
Mouth or nose ulcers 12%


Butterfly Rash


Raynaud’s Phenomenon


Features Characteristics
Malar rash Fixed erythema, flat or raised, sparing the nasolabial folds
Discoid rash Erythematous raised patches with adherent keratotic scarring
and follicular plugging
Photosensitivity Skin rash as a result of unusual reaction to sunlight
Oral ulcers Oral or nasopharyngeal ulceration; may be painless
Arthritis Non-erosive, involving two or more peripheral joints
Serositis a. Pleuritis (convincing history of pleuritic pain, rub or
pleural effusion) or
b. Pericarditis (rub, ECG evidence or effusion)
Renal disorder a. Persistent proteinuria >0.5 g/day, or
b. Cellular casts (red cell, granular or tubular)
Neurological Seizures or psychosis in the absence of offending drugs or
disorder metabolic derangement


Features Characteristics
Haematological a. Haemolytic anaemia or
disorder b. Leucopenia (<4000/mm3) or
c. Lymphopenia (<1500/mm3) or
d. Thrombocytopenia (<10 000/mm3) in the absence of
offending drugs
Immunology a. Anti-DNA antibodies in abnormal titre or
disorder b. Presence of antibody to Sm antigen or
c. Positive antiphospholipid antibodies
Antinuclear Abnormal titre of ANA by immunofluorescence
antibody disorder


Complications (Blood)__________
• Affects 85% of patients with SLE
• 50% of patients have anemia
• Between 34 - 42% of patients have
antiphospholipid syndrome (APS)
• Patients who have APS have a high incidence
of blood clots
• Blood clotting puts patients at higher risk for
stroke and pulmonary embolism

Clot in Deep Veins of Groin


Complications (Heart &
Circulation)
• Heart disease is primary cause of death in lupus
patients
• Atherosclerosis, or plaque buildup in the arteries
• High blood pressure, most likely because of
kidney injury and corticosteroid treatments
• Heart failure
• Pericarditis, an inflammation of the tissue
surrounding the heart
• Myocarditis, an inflammation of the heart muscle
itself (rare)


Complications (Lung)___________
• Affects 60% of SLE patients
• Inflammation of the membrane lining the lung
(pleurisy) is the most common problem, which
can cause shortness of breath and coughing.
• In some cases, fluid accumulates, a condition
called pleural effusion.


Complications (Kidney)__________
• Affects 50% of patients
• Poor kidney function (mild) and kidney failure
(severe) may result from this damage.
• Serious complications occur in 30% of patients


Complications (CNS)____________
• Nearly all patients complain of CNS complications
• Most serious: inflammation of the blood vessels in the
brain (vasculitis), occurs in 10% of patients
• Fever, seizures, psychosis, and even coma can occur.
• Irritability
• Emotional disorders (anxiety, depression)
• Mild impairment of concentration and memory
• Migraine and tension headaches
• Problems with the reflex systems, sensation, vision,
hearing, and motor control

Complications (Other)___
• Infections (common)
• Gastrointestinal (45%)
• Joint, Muscle, Bone (Osteoporosis, Arthritis)
• Eye (5% temporary blindness)


Diagnosis_____________________
• Difficult to diagnose
• No single diagnostic marker; identified through a
combination of clinal and laboratory criteria
• Early diagnosis is important as it reduces morbidity
and mortality (lupus nephritis)


Laboratory Investigations________
• 4 of 11 clinical and laboratory criteria must be met
• Antinuclear antibody titer is the primary laboratory test
• Antinuclear antibody titer of 1:40 and characteristic
multiorgan system involvement can be diagnosed with
systemic lupus erythematosus without additional testing
• Patients with an antibody titer of 1:40 who fail to meet full
clinical criteria should undergo additional testing: including
tests for antibody to doublestranded DNA antigen and
antibody to Sm nuclear antigen.


Treatment and Cure____________
• No cure
• Treatment goal: relieve symptoms and protect organs by
decreasing inflammation and/or the level of autoimmune activity in
the body.
• Treatment options:
 Rest/Sleep
 Nonsteriodal anti-inflammatory drugs (taken with medication to
prevent ulcers)
 Corticosteriods (more potent than NSAIDs in reducing
inflammation)
 Hydroxychloroquine (anti-malarial)
 Cytotoxic drugs (immunosuppressive medications)


Prognosis____________________
_
• Highly variable prognosis for individual
patients
• Relatively benign to rapidly progressive/fatal
cases
• Often waxes and wanes in affected individuals
throughout life, and features of the disease
vary greatly between individuals.


Prevention___________________
_
• There is currently no way to prevent SLE
• But people who smoke may be more likely to
develop lupus.
• Avoiding smoking and perhaps other tobacco
products may decrease your risk of developing lupus.


Systemic Lupus Erythematosus

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