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Systemic Lupus Erythematosus (or SLE for short)

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Name(s)_____________________ _ • Systemic Lupus Erythematosus • Abbreviations: SLS or Lupus • Alternative Names: Disseminated lupus erythematosus; erythematosus; Discoid lupusFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Definition____________________ A chronic inflammatory condition caused by an autoimmune disease. An autoimmune disease occurs when the bodys 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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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Aetiology_____________________ • No definitive cause BUT…Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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 reactionFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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 100,000Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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 fallsFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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 factorsFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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%Find out more about Asthma Treatment Systemic Lupus Erythematosus

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Arthritis (Swollen Joints)Find out more about Asthma Treatment Systemic Lupus Erythematosus

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Presenting Features____________ • 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%Find out more about Asthma Treatment Systemic Lupus Erythematosus

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Butterfly RashFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Raynaud’s PhenomenonFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Presenting Features____________ 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 derangementFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Presenting Features____________ 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 disorderFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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 embolismFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Clot in Deep Veins of GroinFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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)Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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 patientsFind out more about Asthma Treatment Systemic Lupus Erythematosus

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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 controlFind out more about Asthma Treatment Systemic Lupus Erythematosus

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Complications (Other)___ • Infections (common) • Gastrointestinal (45%) • Joint, Muscle, Bone (Osteoporosis, Arthritis) • Eye (5% temporary blindness)Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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)Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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)Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus

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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.Find out more about Asthma Treatment Systemic Lupus Erythematosus