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Systemic lupus erythematosus Causes and Risks:
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 8 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.
The incidence is 4 out of 10,000 people. African Americans and Asians are affected more often than other races.
Prevention:
unknown
Symptoms:
Additional symptoms that may be associated with this disease:
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: This disease may also alter the results of the following tests:
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 (see Corticosteroids - topical - low potency) 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.
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 .
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:
Call Your Healthcare 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.
Lupus, discoid on the face: The discoid (round or disk shaped) rash is characterized by red, raised patches with adherent scales. The skin pores (follicles) may be plugged. Scarring often occurs in older lesions. The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).
Lupus, discoid on a man's face: The discoid (round or disk shaped) rash is characterized by red, raised patches with adherent scales. The skin pores (follicles) may be plugged. Scarring often occurs in older lesions. Lesions are most often found in the sun exposed skin of the face, scalp, and arms. Involvement of the scalp often results in a scarring of the scalp with associated permanent hair loss.
Systemic Lupus Erythematosis: Lupus often produces a "butterfly rash" on the malar rash. This rash is usually red, smooth with no scale or crusts. Patients often have other symptoms like arthritis, fever, and muscle aches.
Lupus, rash on a female's chest: Lupus is a multi-system autoimmune disease that can affect almost any organ of the body. The skin is frequently involved. Rashes may have many different appearances. This is one manifestation seen here on the neck and chest. Lupus rashes most commonly occur in the sun-exposed skin.
Lupus is a multi-system autoimmune disease that can affect almost any organ of the body. The skin is frequently involved. Rashes may have many different appearances. These skin lesions are more characteristic of subacute or discoid lupus than the typical " butterfly rash" of systemic lupus.
Erythema nodosum consists of red to reddish purple hard (indurated) nodules which are painful to the slightest touch. These are most commonly seen on the shins (as pictured), but may occur on the thighs or elsewhere. They often occur in association with a group of widely varied diseases such as tuberculosis (TB), sarcoidosis, coccidioidomycosis, SLE, other fungal infections, and some drugs.
Lupus, discoid - view of lesions on the chest: This close-up picture of the neck clearly shows the typical rounded appearance of discoid lupus. The whitish appearance is caused by scaling. The two dark spots are biopsy sites and are not part of the disease.
Lupus, discoid on the cheek and neck: The rash of discoid lupus may have several different appearances. This rash alone is not diagnostic of discoid lupus.
Erythema nodosum usually occurs in association with specific infections, inflammatory conditions, or medications. It consists of painful, hot, red skin lumps, usually over the shins. People with erythema nodosum should be evaluated for other disease processes.
The round or disk shaped (discoid) rash of lupus produces red, raised patches with scales. The pores (hair follicles) may be plugged. Scarring often occurs in older lesions. The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).
This is a picture of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a "butterfly rash" or malar rash. Typically, the rash also appears on the nose.
The rash of lupis is round or disk shaped (discoid) and is characterized by red, raised patches with adherent scales. The skin pores (follicles) may be plugged, and scarring often occurs in older lesions. Approximately 90% of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).
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