Systemic Lupus Erythematosus an Auto-immune Disease
Systemic Lupus Erythematosus (lupus) is an auto-immune disease that results in episodes of inflammation in joints, tendons, and other connective tissues and organs.
Different tissues and organs become inflamed in different people, and the severity of the disease ranges from mild to debilitating, depending on the number and variety of antibodies that appear and the organs affected. About 90 per cent of the people who have lupus are young women in their late teens to 30s but children, mostly girls, and older men and women can also be affected. Occasionally certain heart drugs (hydral azine, procairamide, and beta-blockers) can cause a lupus-like syndrome that disappears after the drug is discontinued.
Essentials of Diagnosis :
- Occurs predominantly in young women.
- Symptoms and signs referable to multiple organ systems.
- Weakness, malaise, fever, joint pains, and weight loss.
- Erythematous rash on face or other areas exposed to sunlight.
- Anaemia, leukopenia, thrombocytopenia, hyperglobulinemia, and increased ESR.
- LE cells may be demonstrated in blood and other issues.
- Antinuclear Antibodies usually found in blood test.
The number and variety of antibodies that can appear in lupus are greater than those in any other disease, and they - along with other unknown factors - determine which symptoms develop. Therefore, symptoms, and their severity and gravity, vary greatly from person to person. Lupus can be quite mild, or it can be devastating, disabling, or fatal. For example, in people who have antibodies that affect only the skin, the skin symptoms may be mild - protecting the skin from sunlight may even prevent them - or they may be severe and disfiguring. Because symptoms vary greatly, lupus may resemble many other disease. For example, the connective tissue of joints is commonly affected in lupus, and the arthritis that results may resemble epilepsy or some psychological disorders when the brain is affected. Although lupus can be chronic and ongoing, it usually flares up intermittently. What triggers flare-up of lupus in people who are predisposed to it are often is not known, although sunlight seems to be one factor. Lupus may begin with a fever. A high fever can occur abruptly, or episodes of fever and a generally sick feeling (malaise) can come and go some times for years. About 90 per cent of people with lupus have joint inflammation, which ranges from intermittent mild aches to severe arthritis in several joints.
Years of joint symptoms may precede other symptoms. In fact, many people who have lupus recall having growing pains as children. Long-standing joint inflammation can lead to deformity and permanent damage to the joint and surrounding tissue, but the bone does not erode as it does in rheumatoid arthritis. Skin rashes are common, often occurring on the face, neck, upper chest, and elbows. The characteristic is a red, butterfly shaped rash that appears across the bridge of the nose and on the cheeks. Circular, raised bumps may develop.
These rashes rarely blister or become raw. Mouth sores are also common. Mottled, reddish purple areas may appear on the sides of the palms and on the fingers, swelling and redness may develop around the nails. Hair loss is common when the disease is active. In almost, half the people who have lupus, the skin is highly sensitive to light: it may burn easily or a rash may develop after exposure to sunlight.
Occasionally, an inflammation develops and extra fluid accumulates in the membranes surrounding the lungs. This inflammation (pleurisy) can make deep breathing painful. Fluid may accumulate in the sac around the heart, resulting in pericarditis, which can cause severe, constant chest pain. Children young, adults, and blacks with lupus commonly develop swollen lymph nodes throughout the body, and about 10 per cent of the people with lupus develop an enlarged spleen.
Sometimes the nervous system is affected causing headaches, personality changes, seizures, and symptoms that resemble dementia, such as difficulty in thinking clearly. Strokes occur less often. Protein or red blood cells in the urine detected by a laboratory test indicate kidney damage caused by glomerufonephritis, an inflammation of the kidneys, which is a common consequence of lupus.
If severe, progressive kidney disease develops, blood pressure can become dangerously high, and kidney failure, which may be fatal, can follow. Early detection and treatment of kidney damage in people who have lupus reduces the incidence of severe kidney disease.
Besides clinical findings, laboratory tests can help confirm the diagnosis. A blood test can detect antinuclear antibodies, which are present in almost all people who have lupus. However, these antibodies also occur in other disease. Therefore, if antinuclear anti-bodies are detected, a test for antibodies to double stranded DNA is also performed.
A high level of these antibodies is also specific for lupus, but not all people who have lupus have these antibodies. Blood tests to measure complement levels (a group of proteins that are part of the immune system) and to detect other antibodies may be performed to predict the activity and the course of the disease. Kindney damage from lupus may be detected by blood and urine tests. Sometimes a biopsy of kidney tissue must be performed to help the doctor plan treatment.
If the symptoms of lupus are caused by taking a drug, discontinuing the drug cures the lupus, although the recovery may take time. Treatment depends on which organs are affected and whether the disease is mild or severe, Mild lupus is characterised by fever, arthritis, rash, mild heart and lung involvement, and headaches. Severe lupus may cause life threatening blood disorders, massive heart and lung involvement, significant kidney damage, vasculitis of the arms and legs or gastro-intestinal tract, or severe nervous system dysfunction.
Mild disease may not require or may require little treatment such as non-steroidal anti-inflammatory drugs (NSAID) for relief from joint pain. Aspirin may be used in low doses if the patients blood has a tendency to clot. Hydroxychloroquine, chloroquin or quinacrine, sometimes taken in combination help relieve joint and skin symptoms. Severe disease is treated immediately with a corticosteroid. The dose and duration depend on which organs are affected. Sometimes an immuno-suppressive drug such as azathioprine or cyclophosphamide is given to suppress the body's autoimmune attack. For nervous system disease. Kidney disease or vasculitis the combination of a corticosteroid and immune-suppressive drug is most often used.
Prognosis varies widely because the course of lupus is unpredictable. The disease tends to be chronic and relapsing, often with symptom free periods that can last for years. Flare-ups rarely occur after menopause. The prognosis has improved markedly over the last two decades. Usually, if the initial inflammation is controlled, the long term prognosis is good.
Dr. M. R. Bhuiya
- Handout on Health, Systemic Lupus Erythematosus: NIAMS
- Systemic lupus erythematosus - Symptoms: University of Maryland
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