SLE Lupus- A Rare Disease

Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus
(This article is dedicated to our beloved friends)

SLE (lupus) is an autoimmune disease. This means there is a problem with the body's normal immune system response. Normally, the immune system helps protect the body from harmful substances. But in patients with an autoimmune disease, the immune system can't tell the difference between harmful substances and healthy ones. The result is an overactive immune response that attacks otherwise healthy cells and tissue. This leads to chronic (long-term) inflammation.

The underlying cause of autoimmune diseases is not fully known. Some researchers think autoimmune diseases occur after infection with an organism that looks like certain proteins in the body. The proteins are later mistaken for the organism and wrongly targeted for attack by the body's immune system.

SLE may be mild or severe enough to cause death.

SLE affects nine times as many women as men. It may occur at any age, but appears most often in people between the ages of 10 and 50 years. African Americans and Asians are affected more often than people from other races.Statistics demonstrate that lupus is somewhat more frequent in African Americans and people of Chinese and Japanese descent.

It also is known that some women with SLE can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of SLE, suggest that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.

Women with SLE appear to be at increased risk for heart disease (coronary artery disease) according to recent reports. Women with SLE should be evaluated and counseled to minimize risk factors for heart disease, such as elevated blood cholesterol, quitting smoking, high blood pressure, and obesity.

Dozens of medications have been reported to trigger SLE. However, more than 90% of this "drug-induced lupus" occurs as a side effect of one of the following six drugs: hydralazine (used for high blood pressure), quinidine and procainamide (used for abnormal heart rhythms), phenytoin (used for epilepsy), isoniazid ([Nydrazid, Laniazid] used for tuberculosis), d-penicillamine (used for rheumatoid arthritis). These drugs are known to stimulate the immune system and cause SLE. Fortunately, drug-induced SLE is infrequent (accounting for less than 5% of all patients with SLE) and usually resolves when the medications are discontinued.

Symptoms vary from person to person, and may come and go. The condition may affect one organ or body system at first. Others may become involved later. Almost all people with SLE joint pain and most develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees.

General symptoms include:
-Arthritis
-Fatigue
-Fever
-General discomfort, uneasiness or ill feeling (malaise)
-Joint pain and swelling
-Muscle aches
-Nausea and vomitting
-Pleural effusions
-Pleurisy (causes chest pain)
-Psychosis
-Seizures
-Sensitivity to sunlight
-Skin rash -- a "butterfly" rash over the cheeks and bridge of the nose affects about half of those with SLE. The rash gets worse when in sunlight. The rash may also be widespread.
-Swollen glands

Additional symptoms that may be associated with this disease:
-Abdominal pain
-Blood disorders, including blood clots
-Blood in the urine
-Coughing up blood
-Fingers that change colour upon pressure or in the cold
-Hair loss
-Mouth sores
-Nosebleed
-Numbness and tingling
-Red spots on skin
-Skin color is patchy
-Swallowing dificulty
-Visual disturbance

Inflammation of various parts of the heart may occur as pericarditis, endocarditis, or myocarditis. Chest pain and arrhythmias may result from these conditions.

The diagnosis of SLE is based upon the presence of at least four out of eleven typical characteristics of the disease. The doctor will listen to your chest with a stethoscope. A sound called a heart friction rub or pleural friction rub may be heard. A neurological exam will also be performed.

Tests used to diagnose SLE may include:
-Antibody tests, including:
-Antinuclear antibody (ANA) panel
-Anti-double strand (ds) DNA
-Anti-phospholipid antibodies
-Anti-smith antibodies
-CBC to show low white blood cells, hemoglobin, or platelets
-Chest x-ray showing pleuritis or pericarditis
-Kidney biopsy
-Urinalysis to show blood, casts, or protein in the urine
-This disease may also alter the results of the following tests:
-Anti-SSA or -SSB antibodies
-Anti-thyroglobulin antibody
-Anti-thyroid microsomal antibody
-Complement components (C3 and C4)
-Coombs' test - direct
-Cryoglobulins
-ESR
-Rheumatoid factor
-RPR - a test for syphilis
-Serum globulin electrophoresis
-Serum protein electrophoresis

There is no cure for SLE. Treatment is aimed at controlling symptoms. Your individual symptoms determine your treatment.

Mild disease that involves a rash, headaches, fever, arthritis, pleurisy, and pericarditis requires little therapy. Nonsteroidal anti-inflammatory medications (NSAIDs) are used to treat arthritis and pleurisy. Corticosteroid creams are used to treat skin rashes. An anti-malaria drug called hydroxychloroquine) and low dose corticosteroids are sometimes used for skin and arthritis symptoms.

You should wear protective clothing, sunglasses, and sunscreen when in the sun.
Severe or life-threatening symptoms (such as hemolytic anemia, extensive heart or lung involvement, kindney disease, or central nervous system involvement) often require treatment by a rheumatologist and other specialists. Corticosteroids or medications to decrease the immune system response may be prescribed to control the various symptoms. Cytotoxic drugs (drugs that block cell growth) are used to treat people who do not respond well to corticosteroids or who might require long-term use of high doses of corticosteroids.

Patients with SLE who become pregnant are considered "high risk." Women with SLE who are pregnant require close observation during pregnancy, delivery, and the postpartum period. This includes fetal monitoring by the obstetrician during later pregnancy. These women can have an increased risk of miscarriages(spontaneous abortions) and can have flares of SLE during pregnancy.

Lupus antibodies can be transferred from the mother to the fetus and result in lupus illness in the newborn ("neonatal lupus"). This includes the development of low red cell (anemia) and/or white blood cell and platelet counts, and skin rash. Problems can also develop in the electrical system of the baby's heart (congenital heart block).

At last but not the least, individuals with SLE can improve their prognosis by learning about the many aspects of the illness as well as closely monitoring their own health with their doctors.