Systemic Lupus Erythematosus (SLE)
The more serious and potentially fatal form of the chronic autoimmune disease lupus erythematosus that affects many systems of the body. The milder form is discoid lupus erythematosus (DLE). Systemic lupus erythematosus (SLE) is probably not one but several conditions; while many systems of the body may be affected, it is also possible that the disease may affect just the skin. Although typically a disease of girls and young women, it can affect either sex and all age groups without regard to race. The disease commonly waxes and wanes, and its etiology is affected by heredity, autoimmunity, certain drugs, sex hormones, ultraviolet light, and viruses.
SLE accounts for about 70 percent of all lupus patients as well as a higher percentage of pediatric patients. Although SLE typically develops during the 20s, 30s, or 40s, about 15 to 17 percent of patients with systemic lupus first notice symptoms during childhood or adolescence. Most of these symptoms are seen in children 10 years or older; it is extremely rare to see symptoms in children younger than five. (Although there are no definitive data, it has been estimated that between 5,000 and 10,000 children in the United States have SLE.)
The relationship between DLE and SLE is controversial. Between 2 and 20 percent of patients who are first diagnosed with DLE go on to develop SLE. It is not uncommon for typical SLE to go into remission, leaving lesions of chronic DLE. On the other hand, DLE may spontaneously subside, remain constant, worsen, or progress to active SLE after some stress.
The prognosis for patients with SLE depends on which organs are involved; kidney or central nervous system involvement implies a poor prognosis. In most patients the disease is chronic; more than 90 percent of patients survive for at least 10 years.
Symptoms
Typically there is a red scaly rash on the face, affecting the nose and cheeks, arthritis, and progressive kidney damage; the heart, lungs, and brain may also be affected by progressive attacks of inflammation followed by the formation of scar tissue. In the milder form (DLE) only the skin is affected. Patients with lupus often have fever, weakness, fatigue, or weight loss. They may experience muscle aches, loss of appetite, swollen glands, hair loss, or abdominal pain, which can be accompanied by nausea, diarrhea, and vomiting. Sometimes the person’s fingers, toes, nose, or ears will be particularly sensitive to cold and will turn white in cold temperatures, a condition known as Raynaud’s phenomenon.
Diagnosis
SLE can be difficult to diagnose, because no two patients have the same symptoms, which also can be mistaken for those of other diseases. SLE may be serious and life-threatening or very mild. To make diagnosing SLE easier, the American College of Rheumatology has published a list of 11 symptoms that are typically seen in SLE; a patient who has at least four of these symptoms probably has SLE:
• rash across the cheeks and the bridge of the nose
• circular, red raised patches of skin that often occur on the face, neck, or chest
• sensitivity to ultraviolet light
• painless ulcers in the nose or mouth
• arthritis that does not destroy the bones around the joints
• inflammation of the lining around the heart, abdomen, or lungs
• kidney problems, either mild (with no early symptoms) or severe
• neurological disorders, such as seizures or psychosis
• blood problems, such as a low red blood cell count (anemia), a low white blood cell count (leukopenia), or a low platelet count (thrombocytopenia)
• problems with the immune system
• positive test for antinuclear antibodies (ANA), specific proteins that are a hallmark of rheumatic or autoimmune disease
Treatment
Local corticosteroid creams and ointments, or a therapeutic trial of salicylate are used to treat SLE. Immunosuppressive agents are sometimes used, especially among those who have experienced side effects from corticosteroids.
SLE accounts for about 70 percent of all lupus patients as well as a higher percentage of pediatric patients. Although SLE typically develops during the 20s, 30s, or 40s, about 15 to 17 percent of patients with systemic lupus first notice symptoms during childhood or adolescence. Most of these symptoms are seen in children 10 years or older; it is extremely rare to see symptoms in children younger than five. (Although there are no definitive data, it has been estimated that between 5,000 and 10,000 children in the United States have SLE.)
The relationship between DLE and SLE is controversial. Between 2 and 20 percent of patients who are first diagnosed with DLE go on to develop SLE. It is not uncommon for typical SLE to go into remission, leaving lesions of chronic DLE. On the other hand, DLE may spontaneously subside, remain constant, worsen, or progress to active SLE after some stress.
The prognosis for patients with SLE depends on which organs are involved; kidney or central nervous system involvement implies a poor prognosis. In most patients the disease is chronic; more than 90 percent of patients survive for at least 10 years.
Symptoms
Typically there is a red scaly rash on the face, affecting the nose and cheeks, arthritis, and progressive kidney damage; the heart, lungs, and brain may also be affected by progressive attacks of inflammation followed by the formation of scar tissue. In the milder form (DLE) only the skin is affected. Patients with lupus often have fever, weakness, fatigue, or weight loss. They may experience muscle aches, loss of appetite, swollen glands, hair loss, or abdominal pain, which can be accompanied by nausea, diarrhea, and vomiting. Sometimes the person’s fingers, toes, nose, or ears will be particularly sensitive to cold and will turn white in cold temperatures, a condition known as Raynaud’s phenomenon.
Diagnosis
SLE can be difficult to diagnose, because no two patients have the same symptoms, which also can be mistaken for those of other diseases. SLE may be serious and life-threatening or very mild. To make diagnosing SLE easier, the American College of Rheumatology has published a list of 11 symptoms that are typically seen in SLE; a patient who has at least four of these symptoms probably has SLE:
• rash across the cheeks and the bridge of the nose
• circular, red raised patches of skin that often occur on the face, neck, or chest
• sensitivity to ultraviolet light
• painless ulcers in the nose or mouth
• arthritis that does not destroy the bones around the joints
• inflammation of the lining around the heart, abdomen, or lungs
• kidney problems, either mild (with no early symptoms) or severe
• neurological disorders, such as seizures or psychosis
• blood problems, such as a low red blood cell count (anemia), a low white blood cell count (leukopenia), or a low platelet count (thrombocytopenia)
• problems with the immune system
• positive test for antinuclear antibodies (ANA), specific proteins that are a hallmark of rheumatic or autoimmune disease
Treatment
Local corticosteroid creams and ointments, or a therapeutic trial of salicylate are used to treat SLE. Immunosuppressive agents are sometimes used, especially among those who have experienced side effects from corticosteroids.
Tags: Anemia, chronic autoimmune disease lupus erythematosus, discoid lupus erythematosus (DLE), hair loss, red scaly rash on the face, SLE, Systemic lupus erythematosus
Posted in Health and Wellness
