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

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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•HLA type•Immunoregulatory genes•Hormonal levels•Complement levels

Environmental Stimuli•UV exposure•Microbial response•Medication

Autoimmune Proliferation•Hyperactive B-cell/T-cell activation•High ratio of CDA;CD8 T-cells•Defective immune complex clearance •Impaired tolerance

Autoantibody Production

•Apoptosis and self exposure•Self-recognition•Foreign-Ab cross reaction

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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)

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• 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 and/or blue in the cold)

17%

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 disorder

Seizures or psychosis in the absence of offending drugs or metabolic derangement





Features Characteristics

Haematological disorder

a. Haemolytic anaemia orb. Leucopenia (<4000/mm3) orc. Lymphopenia (<1500/mm3) ord. Thrombocytopenia (<10 000/mm3) in the absence of

offending drugs

Immunology disorder

a. Anti-DNA antibodies in abnormal titre orb. Presence of antibody to Sm antigen or c. Positive antiphospholipid antibodies

Antinuclear antibody disorder

Abnormal titre of ANA by immunofluorescence



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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