| Subject | Contents |
| Definition | A chronic, inflammatory autoimmune disorder that may affect many organ systems including the skin, joints, and internal organs. |
| Alternative Names | Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus |
| Causes, incidence, and risk factors | Normally the immune system controls the body's defenses against infection. In systemic lupus erythematosus (SLE) and other autoimmune diseases , these defenses are turned against the body when antibodies are produced against its own cells. These antibodies fight against the body's blood cells, organs, and tissues, causing chronic diseases. The mechanism or cause of autoimmune diseases is not fully known. The disease affects 9 times as many women as men. It may occur at any age, but appears mostly in people between the ages of 10 and 50 years. SLE may also be caused by certain drugs. When this occurs, it is known as drug-induced lupus erythematosus and is usually reversible when the medication is stopped. The course of the disease may vary from a mild episodic illness to a severe fatal disease. Symptoms also vary widely with the individual and are characterized by remissions and exacerbation. At its onset, only 1 organ system may be involved. Additional organs may become involved later. The following organ system manifestations may be seen, but other manifestations are possible. Musculoskeletal Almost all people with SLE have joint pain and most develop arthritis . Frequently affected joints are the fingers, hands, wrists, and knees. Death of bone tissue can occur in the hips and shoulders and is frequently a cause of pain in those areas. Skin A malar "butterfly" rash over the cheeks and bridge of the nose affects about half of those with SLE. The rash is usually worsened by sunlight. A more diffuse rash may appear on other body parts that are exposed to the sun. Other skin lesions or nodules can occur. Kidney Most people with SLE have some deposits of protein within the cells (glomeruli) of the kidney; however, only 50% have lupus nephritis as defined by persistent inflammation in the kidney. They may eventually develop renal failure and require dialysis or kidney transplantation. Nervous system Neurologic disorders can affect up to 25% of those with SLE. Mild mental dysfunction is the most common symptom, but any area of the brain, spinal cord, or nervous system can be affected. Seizures , psychosis , organic brain syndrome , and headaches are some of the varied nervous system disorders that can occur. Blood Blood disorders can affect up to 85% of those with SLE. Venous or arterial blood clots can form and are associated with strokes and pulmonary embolism . Often platelets are decreased, or antibodies are formed against blood clotting factors, which may cause significant bleeding (see Lupus anticoagulant ). Anemia of chronic disease often develops at some point in the course of the disease. Heart Inflammation of various parts of the heart may occur as pericarditis , endocarditis , or myocarditis . Chest pain and arrhythmia 's may result from these conditions. Lungs Pleurisy , an inflammation of the lining of the lung, and pleural effusions , a fluid collection between the lung and its lining can occur as a result of SLE or infection. Chest pain and shortness of breath are frequently results of these disorders. African Americans and Asians are affected more often than other races. |
| Symptoms | feverfatigue general discomfort, uneasiness, or ill feeling ( malaise )weight lossskin rash malar "butterfly" rash sunlight aggravates rash '>skin rash sensitivity to sunlightjoint pain and swellingarthritisswollen glandsmuscle achesnausea and vomiting pleuritic chest painseizurespsychosis Additional symptoms that may be associated with this disease: blood in the urinecoughing up bloodnosebleed - symptomswallowing difficulty skin color is patchy red spots on skin fingers that change color upon pressurenumbness and tinglingmouth soreshair lossabdominal pain visual disturbance |
| Signs and tests | The diagnosis of SLE is based upon the manifestations of at least 4 out of 11 typical characteristics of the disease. Tests to determine the presence of these disease manifestations may vary but will include some of the following:antinuclear antibody (ANA) panel characteristic skin rash or lesionschest X-ray showing pleuritis or pericarditis listening to the chest with a stethoscope to reveal heart friction rub or pleural friction rub urinalysis to show blood, casts , or protein in the urineCBC showing a decrease in some cell types kidney biopsy neurological examination This disease may also alter the results of the following tests:WBC countserum globulin electrophoresisrheumatoid factorprotein, urineprotein electrophoresis - serummononucleosis spot testESRcryoglobulinsCoombs' test, directcomplement component 3 (C3) complementantithyroid microsomal antibodyantithyroglobulin antibodyantimitochondrial antibodyanti-smooth muscle antibody |
| Treatment | OVERVIEW: The disease has multiple manifestations with variable severity, which determines individual treatment. There is no cure for SLE. MEDICATIONS: Mild disease ( rash , headaches , fever , arthritis , pleurisy , pericarditis ) requires little therapy. Nonsteroidal anti-inflammatory medications (NSAIDS) are used to treat arthritis and pleurisy. Corticosteroid creams are used to treat skin rashes . Antimalarial drugs (hydroxychloroquine) are sometimes used for skin and arthritis symptoms. Sensitivity to light is treated by protective clothing, sunglasses, and sunscreen. Severe or life-threatening manifestations ( hemolytic anemia , extensive heart or lung involvement, kidney disease , central nervous system involvement) often requires treatment by specialists in the specific area. Corticosteroid therapy or medications to suppress the immune system may be prescribed to control the various manifestations of severe disease. Some health care professionals use cytotoxic drugs (drugs that block cell growth) in people who do not have a good response to corticosteroids or who are dependent on high doses of corticosteroids. LIFESTYLE CHANGES: The stress of illness can often be helped by joining a support group where members share common experiences and problems. See lupus - support group . |
| Support Groups | |
| Expectations (prognosis) | The outcome for people with SLE has improved over recent years. Many of those affected have mild illness. Women with SLE who become pregnant are often able to carry the pregnancy safely to term and deliver normal infants, provided severe renal or heart disease is not present, and the SLE is under treatment. The 10-year survival rate exceeds 85%. People with severe involvement of the brain, lungs, heart, and kidney have the worst prognosis in terms of overall survival and disability. |
| Complications | infection renal failurethrombocytopeniahemolytic anemiamyocarditisseizures |
| Calling your health care provider | Call your health care provider if symptoms of SLE are present. Also, call if you have SLE and symptoms worsen, or if new symptoms develop. |
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