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Description-
Lupus is a chronic, autoimmune disease which causes inflammation of various parts of the body, especially the skin, joints, blood and kidneys. The body's immune system normally makes proteins called antibodies to protect the body against viruses, bacteria and other foreign materials. These foreign materials are called antigens. In an autoimmune disorder such as lupus, the immune system loses its ability to tell the difference between foreign substances (antigens) and its own cells and tissues. The immune system then makes antibodies directed against "self." These antibodies, called "auto-antibodies," react with the "self" antigens to form immune complexes. The immune complexes build up in the tissues and can cause inflammation, injury to tissues, and pain.More people have lupus than AIDS, cerebral palsy, multiple sclerosis, sickle-cell anemia and cystic fibroses combined. LFA market research data show that between 1,400,00 and 2,00,00 people reported to have been diagnosed with lupus. (Study conducted by Bruskin/Goldring Research, 1994.) For most people, lupus is a mild disease affecting only a few organs. For others, it may cause serious and even life-threatening problems. Thousands of Americans die each year from lupus-related complications. For more information on What is Lupus? tryPathology of Systemic Lupus Erythematosus, or Refer to Lupus Home Page., and What is Lupus?.
The cause(s) of lupus is unknown, but environmental and genetic factors are involved. While scientists believe there is a genetic predisposition to the disease, it is known that environmental factors also play a critical role in triggering lupus. Some of the environmental factors that may trigger the disease are: infections, antibiotics (especially those in the sulfa and penicillin groups), ultraviolet light, extreme stress, and certain drugs. Although lupus is known to occur within families, there is no known gene or genes which are thought to cause the illness. Only 10 percent of lupus patients will have a close relative (parent or sibling) who already has or may develop lupus. Statistics show that only about 5% of the children born to individuals with lupus will develop the illness. Lupus is often called a "woman's disease" despite the fact that many men are affected. Lupus can occur at any age, and in either sex, although it occurs 10-15 times more frequently among adult females than among adult males. The symptoms of the disease are the same in men and women. People of African, American Indian, and Asian origin are thought to develop the disease more frequently than Caucasian women, but the studies that led to this result are small and need corroboration. Hormonal factors may explain why lupus occurs more frequently in females than in males. The increase of disease symptoms before menstrual periods and/or during pregnancy support the belief that hormones, particularly estrogen, may be involved. However, the exact hormonal reason for the greater prevalence of lupus in women, and the cyclic increase in symptoms, is unknown.For further information on the Role of autoantibodies produced by the immune system as a trigger reffer to Antiphospholipid Antibodies And Systemic Lupus Erythematosus.For information on direct influence of lupus on the immune system refer to Lupus And Infections and Immunizations.
TYPE 1:DISCOID LUPUS .
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Discoid lupus is always limited to the skin. It is identified by a rash that may appear on the face, neck and scalp. Discoid lupus is diagnosed by examining a biopsy of the rash. In discoid lupus the biopsy will show abnormalities that are not found in skin with the rash. Discoid lupus does not generally involve the body's internal organs. Therefore, the ANA test, a blood test used to detect systemic lupus, may be negative in patients with discoid lupus. However, in a large number of patients with discoid lupus, the ANA test is positive, but at a low level or "titer."n approximately 10 percent of the people with lupus, discoid lupus can evolve into the systemic form of the disease, which can affect almost any organ or system of the body. This cannot be predicted or prevented. Treatment of discoid lupus will not prevent its progression to the systemic form. Individuals who progress to the systemic form probably had systemic lupus at the outset, with the discoid rash as their main symptom For more information on dermatologic manifestations of Discoid type Lupus refer to Pathology of Systemic Lupus erythematosus.or reer to Skin Diseases in Lupus.
The major hematological symptoms of SLE are: anemia, thrombocytopenia (low platelet count), clotting disturbances, and low white blood cell counts.Each of these are described briefly, for detailed information on Blood disorders in SLE refer to Blood Disorders in SLE.
ANEMIA is the most common hematological abnormality in SLE, characterized by a reduction in the number of red blood cells.Although it is not a specific condition, it has many causes. In a person with lupus, anemia may be caused by chronic inflammation, prolonged uremia, iron deficiency, or hemolytic anemia. Inflammation is an important cause of anemia in lupus patients. Prolonged inflammation hinders the production of red blood cells by the bone marrow. Iron, which is essential for the production of hemoglobin,is not handled normally during inflammation and accumulates unused in the marrow tissue.
THROMBOCYTOPENIA is a deficiency of platelets,that leads to excessive bruising of the skin or bleeding from the gums, nose, or intestines. The most common cause of thrombocytopenia in persons with lupus is immune thrombocytopenia. Often referred to as "ITP", this is caused by antibodies against platelets. The antibodies destroy the platelets in a manner similar to the destruction of red blood cells in autoimmune hemolytic anemia. ITP is usually treated with steroids (Prednisone), but a splenectomy may be necessary in some cases. During pregnancy, anti-platelet antibodies can cross the placenta, enter the baby's blood and cause thrombocytopenia.
GRANULOCYTOPENIA and Lymphocytopenia refer to the redution in either of the two principal types of white blood cells (granulocytes and lymphocytes),causing granulocytopenia (low granulocyte count) and lymphocytopenia (low lymphocytes count). Low serum WBC's nay result in immunodeficiency.
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Vasculitis is an inflammation of the blood vessels,in which tissue is damaged by blood cells entering the tissues. These are mostly white blood cells which circulate and serve as our major defense against infection. Ordinarily, white blood cells destroy bacteria and viruses. However, they can also damage normal tissue if they invade it. Vasculitis can affect very small blood vessels (capillaries), medium size blood vessels (arterioles or venules), or large blood vessels (arteries or veins).
Several things can happen to an inflamed blood vessel. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. If a larger vessel is inflamed, it may swell and produce a nodule. The inside of the vessel tube may become narrowed so that blood flow is reduced, or the inside may become totally closed (usually by a blood clot which forms at the site of inflammation). If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die. For example, a person with vasculitis of a medium sized artery in the hand may develop a cold finger which hurts whenever it is used; occasionally this can progress to gangrene. For further information on the Causes, Diagnosis and Treatment of Vasculitis refer to Lupus and Vasculitis
The heart and the lungs are frequently affected in patients with Systemic Lupus Erythematosus (SLE). The degree of cardiopulmonary involvement ranges from no symptoms to life-threatening complications. In fact, heart disease is the third most common cause of death in people with SLE CARDIAC (HEART) INVOLVEMENT. Lupus can involve all parts of the heart including; the pericardium (sac surrounding the heart), myocardium (muscle layer), endocardium (lining of the inside of the heart) and the coronary arteries.
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When lupus causes inflammation of the myocardium, myocarditis occurs. The symptoms of myocarditis include: an unexplained rapid heart beat, an abnormal electrocardiogram, an irregular heart beat and heart failure. Myocarditis is frequently associated with inflammation of other muscles in the body. Treatment of lupus myocarditis usually includes corticosteroids. Immunosuppressive drugs (Cytoxan, Imuran) may be added if the inflammation is not completely controlled with steroids. Myocarditis can lead to tissue damage and replace heart tissue with scar tissue.
Pleuritis (pleurisy) is the most common pulmonary manifestation of SLE. When this membrane is attacked by autoantibodies and becomes inflamed, it is called pleuritis. Sometimes, an excess amount of fluid can accumulate in the pleural space. Symptoms of pleuritis include severe, often sharp, stabbing pain that may be pin-pointed to a specific area or areas of the chest. The pain is often made worse by taking a deep breath, coughing, sneezing or laughing. Analgesics, non-steroidal anti-inflammatory drugs, and/or corticosteroids may be used to treat pleuritis. Pleural effusions will usually respond to these medications or clear by themselves with time.For more information on Cardiopulmonary involvment in Lupus refer to Cardiopulmonary Disease in Lupus.
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There are very few signs or symptoms of lupus nephritis. It does not produce pain in the abdomen or back, nor does it cause pain or burning during urination. The loss of protein in the urine from lupus nephritis may lead to fluid retention with weight gain and swelling (edema). This may result in puffiness in the legs, ankles and/or fingers. This puffiness is often the first symptom of lupus nephritis noted by the patient.
Despite appropriate treatment, some patients with lupus nephritis develop progressive loss of kidney function and renal failure.
For information on implications in kidney disease refer to Kidney Disease and Lupus.
Drug-induced lupus occurs after the use of certain prescribed drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus. The drugs most commonly connected with drug-induced lupus are hydralazine (used treat high blood pressure or hypertension) and procainamide (used to treat irregular heart rhythms). However, not everyone who takes these drugs will develop drug-induced lupus. Only about 4 percent of the people who take these drugs will develop the antibodies suggestive of lupus. Of those 4 percent, only an extremely small number will develop overt drug-induced lupus. The symptoms usually fade when the medications are discontinued.
Because many symptoms of systemic lupus erythematosus (SLE) mimic those of other illnesses, lupus can be a difficult disease to diagnose. Diagnosis is usually made by a careful review of three factors: · the patient's entire medical history; · the patient's current symptoms; and · an analysis of the results obtained in routine laboratory tests and some specialized tests related to immune status. To make a diagnosis of SLE, an individual must show clinical evidence of a multi-system disease (i.e. has shown abnormalities in several different organ systems). The following are typical manifestations which might lead to suspicion of SLE.
Location | Observations |
|---|---|
| Skin | butterfly rash; ulcer in mouth; hair loss |
| Joints | pain; redness and swelling |
| Kidney | abnormal urinanalysis |
| Lining Membranes | pleurisy; pericarditis |
| Blood | hemoluytic anemia |
| Lungs | infiltrates |
| Nervous System | convulsions; psychosis |
Antimalarial Drugs
Anti-Inflammatory Drugs
Corticosteroids
Chemotherapy
For a complete detailed information on treatments refer to Medications Used in The Diagnosis of Lupus
Martindale;s Health Scince Guide
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