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Systemic Lupus Erythematosus

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of Lupus.

Alternative Names

Corticosteroids; Immunosuppressant Drugs

Complications

Systemic lupus erythematosus (SLE) is one of the most serious rheumatic diseases. According to a 2002 government study, the annual number of deaths has risen from 879 to 1,406 since 1979. About third of these deaths occur in people aged 15 to 44 years, mostly women. (Such numbers may be underestimates, since SLE can affect so many organs that a cause of death in some people with SLE may not have been directly attributed to the condition.) It is important to note that a primary cause of death among patients with lupus is atherosclerosis, disease of the coronary blood vessels resulting from accelerated buildup of plaque.

SLE is unpredictable and varies greatly form one individual to the next. Severity also appears to differ among ethnic groups and countries. In Europe and North American SLE patients for example, overall five-years survival rates are between 93% and 95%, while in Asia or Africa they are considerable lower (60% to 70%). Other research also indicates that in the US African American and Hispanic patients suffer greater organ damage than Caucasian patients. Genetic factors appear to have some influence on specific effects of SLE on organ damage among ethnic groups. The poorer outlook among minority groups and in underdeveloped nations appears to be primarily due to less access to good health care.

Mild SLE. About 20% to 30% of cases are mild. For many of these patients, the only symptoms may be the skin rashes of discoid lupus erythematosus (DLE) or subacute cutaneous lupus erythematosus (SCLE) with or without joint aches. The number and intensity of symptoms in mild cases often decrease over time, as does the likelihood of major organ involvement. These skin conditions, however, are not absolute insurance against more severe disease, and patients with mild SLE should be tested for organ involvement.

Widespread SLE. Most commonly, SLE is a chronic, life-long disease, alternating between periods of symptom-relapse, or flares, and remission. The disease may begin in any of the various systems of the body and progress unpredictably to others. The following are typical patterns:

  • Symptom relapses, or flares, occur on the average of two or three times a year.
  • Between flares, most SLE patients can function at about 90% of normal capacity.

The degree of severity depends on different factors.

  • Severity of the inflammatory response.
  • Frequency of episodes.
  • The degree of organ or system involvement. Vital organs or systems, such as lungs, kidneys, nervous system, joints skin, and others are affected in 50% to 75% of SLE patients. Infections followed by kidney failure are the chief causes of death in SLE patients.

Because of more effective and aggressive treatment, the prognosis for SLE has improved markedly over the past two decades. Long-term progress of the disease is affected greatly by treatment in the initial acute phase of the disease so a speedy and accurate diagnosis is all-important. The 10-year survival rate with treatment is now 85% to 95% and many people have a normal life span. SLE that develops later in life is generally less serious than SLE that strikes in childhood.

Complications of the Blood

Almost 85% of SLE patients experience problems associated with abnormalities in the blood.

Anemia. About half of SLE patients are anemic. Causes include the following:

  • Iron deficiencies resulting from excessive menstruation.
  • Iron deficiencies from GI bleeding caused by some of the treatments.
  • A specific anemia called hemolytic anemia, which destroys red blood cells, can occur with very high levels of the anticardiolipin antibody. It can be chronic or develop suddenly and severely.

The Antiphospholipid Syndrome. Between 34% and 42% of SLE patients have the antiphospholipid syndrome (APS). This is a specific set of conditions related to the presence of autoantibodies called lupus anticoagulant and anticardiolipin. These autoantibodies react against fat molecules called phospholipids, and so are called antiphospholipids. Their actions have complex effects that include causing narrowing and abnormalities of blood vessels.

Conditions most often associated with APS include the following:

  • Patients who have APS have a very incidence of blood clots, which most often occur in the deep veins in the legs (32%). Blood clotting in turn puts patients at higher risk for stroke (13%) and pulmonary embolism (clots in the lungs) (9%).
Deep venous thrombosis, ileofemoral
This picture shows a red and swollen thigh and leg caused by a blood clot (thrombus) in the deep veins in the groin (ileofemoral veins) which prevents normal return of blood from the leg to the heart.
  • About 22% of patients have thrombocytopenia--a reduction in blood platelets, which can cause bleeding.
  • The effects on blood vessels have also been associated with confusion, headaches, and seizures. Leg ulcers can also develop.
  • Patients with APS who become pregnant have a high incidence of pregnancy loss, especially in the late term.

Not all patients with APS carry both of the autoantibodies, and they can also wax and wane and so have varying effects. It should also be noted that APS occurs without lupus in about half of those with the syndrome.

Thrombocytopenia. In thrombocytopenia, antibodies attack blood platelets. In such cases, blood clotting is impaired, causing bruising, bleeding from the skin, nose, gums, or intestines. (This condition can also occur in APS, but it is not considered to be one of the standard features of the syndrome.)

Neutropenia. Commonly, patients with SLE have low counts of white blood cells (a condition called neutropenia), but the condition is usually harmless unless the reductions are so severe that they leave the patient vulnerable to infections.

Acute Lupus Hemophagocyte Syndrome. A rare blood complication of SLE that occurs primarily in Asians is called acute lupus hemophagocytic syndrome. It is generally of short duration and characterized by fever and a sudden drop in blood cells and platelets.

Raynaud's Phenomenon

Raynaud's phenomenon is a condition in which cold or stress can cause spasms in impaired blood vessels resulting in pain in fingers and toes. It occurs as part of the inflammatory response in blood vessels, which can narrow them and reduce circulation. In extreme cases, gangrene can result.

Raynaud's phenomenon Click the icon to see an image of Raynaud's phenomenon.

Heart and Circulation Complications

Cardiovascular disease is a primary cause of death in lupus patients. The immune response in SLE can cause inflammation and other damaging effects that can cause significant injury to the arteries and tissues associated with the circulation and the heart. In addition, SLE treatments (particularly corticosteroids) affect cholesterol, weight, and other factors that can also affect the heart. For decades experts have questioned the extent to which the drugs used to treat SLE were contributing to the high rate of atherosclerosis in such patients. Numerous studies now suggest that something about the disease process itself, possibly the chronic inflammation of the blood vessels, probably lies at the root of this dangerous problem. In any event, SLE patients, have a higher chance for the following conditions, which put them at risk for heart attack or stroke:

  • Atherosclerosis, or plaque buildup in the arteries.
  • Increased stiffness in the arteries.
  • Unhealthy cholesterol and lipid (fatty molecules) levels.
  • High blood pressure, most likely because of kidney injury and corticosteroid treatments.
  • Congestive heart failure.
  • Pericarditis, an inflammation of the tissue surrounding the heart that causes pressure or pain in the chest. Occurs in about 30% of patients. Inflammation of the heart muscle itself (myocarditis), although uncommon, can lead to irregular heartbeats or even heart failure.
Pericarditis Click the icon to see an image of pericarditis.
  • Abnormalities in the valves of the heart. (This is less common but can occur in SLE.)
  • Elevated levels of homocysteine, which occurs with deficiencies of vitamins B6, B12, and folic acid. Homocysteine is now a strong suspect in heart attack, strokes, and blood clots.
  • Blood clots.

The risk for cardiovascular disease, heart attack and stroke is much higher than average in younger women with SLE; the risks decline as such women age.

Lung Complications

SLE affects the lungs in about 60% of patients:

  • Recurrent inflammation of the membrane lining the lung (pleurisy) is the most common problem.
  • In some cases, fluid accumulates, a condition called pleural effusion, and can cause stabbing localized pain that worsens when coughing, sneezing, laughing, or taking a deep breath.
  • Inflammation of the lung itself in SLE is called lupus pneumonitis. It can be caused by infections or by the SLE inflammatory process. Symptoms are the same in both cases: fever, chest pain, labored breathing, and coughing. Rarely, lupus pneumonitis becomes chronic and causes scarring in the lungs, which reduces their ability to deliver oxygen to the blood.
  • A very serious and also rare condition called pulmonary hypertension occurs when high pressure develops in the vessels supplying blood to the lungs.
Primary pulmonary hypertension Click the icon to see an image of primary pulmonary hypertension.

Kidney Complications (Lupus Nephritis)

The kidneys are a crucial battleground in SLE because it is here that the debris left over from the immune attacks is most likely to be deposited. About 50% of SLE patients exhibit inflammation of the kidneys (called lupus nephritis).This condition occurs in different forms and can vary widely in severity.

Kidney anatomy Click the icon to see an image of the kidney.
  • Proliferative nephritis is a serious variant of lupus nephritis. It occurs when the inflammatory process causes widespread damage and scarring in the blood vessels of the kidneys, which filters waste products, water, and salts out of the blood. The condition is associated with high blood pressure and kidney deterioration.
  • Membranous lupus nephritis is another variant that is often associated with a good outlook. In some cases, however, if the kidney is persistently exposed to high protein levels, the disorder can progress to fatal end-stage kidney (renal) disease.

Serious complications occur eventually in about 30% of patients. If kidney injury develops, it almost always occurs within 10 years of the onset of SLE, rarely after that.

Central Nervous System Complications

Nearly all SLE patients report some symptoms relating to problems that occur in the central nervous system (CNS), which includes the spinal cord and the brain. Most of these symptoms are minor and some, such as headache, may be related to depression rather than the disease itself. CNS involvement is more likely to occur in the first year, usually during flare-ups in other organs. Symptoms vary widely and may be indistinguishable from psychiatric or neurologic disorders or from the side effects of some medications used for SLE. They include the following:

  • Irritability.
  • Emotional disorders (anxiety, depression).
  • Mild impairment of concentration and memory.
  • Headache. Both migraine and tension headaches are common, although most likely an emotional component associated with the disease is responsible for headache pain rather than SLE itself.
  • Occasionally, the reflex systems, sensation, vision, hearing, and motor control can be affected.
  • The most serious CNS disorder is inflammation of the blood vessels in the brain, which occurs in 10% of SLE patients. Fever, seizures, psychosis, and even coma can occur.

Central nervous system symptoms are usually transient and mild, although, unfortunately, there is little effective treatment available for them. The severity of CNS symptoms correlates with progression of the disease.

Infections

Infections are a common complication and a major cause of death in all stages of SLE. The immune system is indeed overactive in SLE, but it is also abnormal and reduces the ability to fight infections. Patients are not only prone to the ordinary streptococcal and staphylococcal infections, but they are also susceptible to fungal and parasitic infections (called opportunistic infections), which are common in people with weakened immune systems. They also face an increased risk for herpes, salmonella, and yeast infections. Corticosteroid and immunosuppressants, treatments used for SLE, also increase the risk for infections, thereby compounding the problem.

Gastrointestinal Complications

About 45% of SLE patients suffer gastrointestinal problems, including nausea, weight loss, mild abdominal pain, and diarrhea. Severe inflammation of the intestinal tract occurs in less than 5% of patients and causes acute cramping, vomiting, diarrhea, and, rarely, intestinal perforation, which can be life-threatening. Fluid retention and swelling can cause intestinal obstruction, which is much less serious but causes the same type of severe pain. Inflammation of the pancreas can be caused by the disease and by corticosteroid therapy.

Joint, Muscle, and Bone Complications

Arthritis caused by SLE almost never leads to destruction or deformity of joints. The inflammatory process can, however, damage muscles and cause weakness. SLE patients also commonly experience reductions in bone mass density (osteoporosis) and have a higher risk for fractures, whether or not they are taking corticosteroids (which are known risk factors for osteoporosis).

Osteoporosis Click the icon to see an image of osteoporosis.

Eye Complications

Inflamed blood vessels in the eye can reduce blood supply to the retina, resulting in degeneration of nerve cells and a risk of hemorrhage in the retina. The most common symptoms are cotton-wool-like spots on the retina. In about 5% of patients sudden temporary blindness may occur.

Socioeconomic Consequences

In one study, 40% of SLE patients quit work within four years of diagnosis, and many had to modify their work conditions. Significant factors that predicted job loss included high physical demands from the work itself, a more severe condition at the time of diagnosis, and lower educational levels. People with lower income jobs were at particular risk for leaving them.

Pregnancy and Systemic Lupus Erythematosus

In some studies 6% to 15% of women report fewer flares during pregnancy. Most flares occur during the first or second trimester and two months after delivery. Women who conceive after at least six months of remission have a lower risk for flares.

Effect of SLE During and After Pregnancy

All lupus pregnancies are regarded as high risk. Evidence has suggested that 75% of pregnancies are carried to term, although 25% of the babies may be premature. (Newer treatments may be significantly improving these rates.)

Miscarriage. About 25% of SLE pregnancies result in miscarriage. The risk for miscarriage is highest in patients with one or more of the following conditions:

  • Women who have antiphospholipid antibodies that cause blood clotting problems.
  • Women who have active kidney disease.
  • Women with hypertension.

Bleeding in Pregnant Woman. There is an increased risk for bleeding problems after the birth, due to either anti-SLE drugs or SLE itself.

Preeclampsia. Preeclampsia, a dangerous condition associated with high blood pressure that occurs during pregnancy, develops in 20% of pregnant SLE women.

Pulmonary Hypertension. In this condition, blood pressure in the lungs increases, which can be life-threatening. It is not common in SLE pregnancies but some cases have been reported.

Managing SLE During Pregnancy

Many anti-SLE drugs are safe during pregnancy, although caution is advised with antimalarial and immunosuppressant drugs, and cyclophosphamide should always be avoided.

Women with antiphospholipid syndrome (APS) are usually treated with prednisone and aspirin Investigators were also studying combination of aspirin and standard heparin (a major blood-thinning agent). A 2002 study suggested, however, that low-dose aspirin was just as effective the combination or heparin alone. Experts reviewing the study recommended avoiding heparin if possible. A newer form of heparin called low-molecular-weight heparin (LMWH), which includes enoxaparin (Lovenox), dalteparin (Fragmin), and tinzaparin (Innohep), is proving to be beneficial and safer than standard heparin. Investigators are also testing intravenous immunoglobulin, which is showing excellent results. The optimal treatment is still in question, although a 2003 study suggested that LMWH plus aspirin, which achieved an 84% life-birth rate, may be the best option at this time for many women.

One study indicated that any pregnant women with SLE should be treated with heparin. In the study, when all pregnant SLE women were treated with heparin, the infant survival rate was 93% compared to only 77% when heparin was given only to women with APS.

Dangers to the Newborn

Thrombocytopenia. During pregnancy anti-phospholipid antibodies may also cross the placenta and cause thrombocytopenia (drop in platelets) in the fetus, although babies with low-platelet counts are nearly always delivered safely.

Neonatal Lupus. Another known risk to the baby is neonatal lupus, which occurs in 3% of SLE pregnancies and usually resolves within six months.

Heart Abnormalities. Rarely, a permanent heart abnormality can occur with this condition, but it is treatable.

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