What is Lupus?

photo © St Thomas' Lupus Trust

Lupus is an autoimmune disease where the body's immune system becomes hyperactive and attacks normal, healthy tissue. This results in symptoms such as inflammation, swelling, and damage to joints, skin, kidneys, blood, the heart, and lungs.

Under normal function, the immune system makes proteins called antibodies in order to protect and fight against antigens such as viruses and bacteria. Lupus makes the immune system unable to differentiate between antigens and healthy tissue. This leads the immune system to direct antibodies against the healthy tissue - not just antigens - causing swelling, pain, and tissue damage.
(* An antigen is a substance capable of inducing a specific immune response.)

What are the different types of lupus?

Several different kinds of lupus have been identified, but the type that we refer to simply as lupus is known as systemic lupus erythematosus or SLE. Other types include discoid (cutaneous), drug-induced, and neonatal.

photo © St Thomas' Lupus Trust

Patients with discoid lupus have a version of the disease that is limited to the skin. It is characterized by a rash that appears on the face, neck, and scalp, and it does not affect internal organs. Less than 10% of patients with discoid lupus progress into the systemic form of the disease, but there is no way to predict or prevent the path of the disease.

SLE is more severe than discoid lupus because it can affect any of the body's organs or organ systems. Some people may present inflammation or other problems with only skin and joints, while other SLE sufferers will see joints, lungs, kidneys, blood, and/or the heart affected. This type of lupus is also often characterized by periods of flare (when the disease is active) and periods of remission (when the disease is dormant).

Drug-induced lupus is caused by a reaction with certain prescription drugs and causes symptoms very similar to SLE. The drugs most commonly associated with this form of lupus are a hypertension medication called hydralazine and a heart arrhythmia medication called procainamide, but there are some 400 other drugs that can also cause the condition. Drug-induced lupus is known to subside after the patient stops taking the triggering medication.

A rare condition, neonatal lupus occurs when a mother passes autoantibodies to a fetus. The unborn and newborn child can have skin rashes and other complications with the heart and blood. Usually a rash appears but eventually fades within the first six months of the child's life.

Who is affected by lupus?

According to the Lupus Foundation of America (LFA), 1.5 to 2 million Americans have some form of lupus. The prevalence is about 40 cases per 100,000 persons among Northern Europeans and 200 per 100,000 persons among blacks. Although the disease affects both males and females, women are diagnosed 9 times more often than men, usually between the ages of 15 and 45. African-American women suffer from more severe symptoms and a higher mortality rate.

Other risk factors include exposure to sunlight, certain prescription medications, infection with Epstein-Barr virus, and exposure to certain chemicals.

What causes lupus?

Although doctors are do not know exactly what causes lupus and other autoimmune diseases, most believe that lupus results from both genetic and environmental stimuli.

Since lupus is known to occur within families, doctors believe that it is possible to inherit a genetic predisposition to lupus. There are no known genes, however, that directly cause the illness. It is probable that having an inherited predisposition for lupus makes the disease more likely only after coming into contact with some environmental trigger.

The higher number of lupus cases in females than in males may indicate that the disease can be triggered by certain hormones. Physicians believe that hormones such as estrogen regulate the progression of the disease because symptoms tend to flare before menstrual periods and/or during pregnancy.

Certain environmental factors have been known to cause lupus symptoms. These include:

• Extreme stress
• Exposure to ultraviolet light, usually from sunlight
• Smoking
• Some medications and antibiotics, especially those in the sulfa and penicillin groups
• Some infections, such as cytomegalovirus (CMV), parvovirus (such as fifth disease), hepatitis C infections, and the Epstein-Barr virus (in children)
• Chemical exposure to compounds such as trichloroethylene in well water and dust

What are the symptoms of lupus?

Since no two cases of lupus are exactly alike, there is a wide range of symptoms that are known to affect many parts of the body. Sometimes symptoms develop slowly or appear suddenly; they can be mild, severe, temporary, or permanent. Most people with lupus experience symptoms in only a few organs, but more serious cases can lead to problems with kidneys, the heart, the lungs, blood, or the nervous system.

Lupus episodes, or flares, are usually noted by a worsening of some of the following symptoms:

• Achy joints (arthralgia), arthritis, and swollen joints, especially in wrists, small joints of the hands, elbows, knees, and ankles
• Swelling of the hands and feet due to kidney problems
• Fever of more than 100 degrees F (38 degrees C)
• Prolonged or extreme fatigue
• Skin lesions or rashes, especially on the arms, hands, face, neck, or back
• Butterfly-shaped rash (malar rash) across the cheeks and nose
• Anemia (oxygen carrying deficiency of red blood cells)
• Pain in the chest on deep breathing or shortness of breath
• Sun or light sensitivity (photosensitivity)
• Hair loss or alopecia
• Abnormal blood clotting problems
• Raynaud's phenomenon: fingers turn white and/or blue or red in the cold
• Seizures
• Mouth or nose ulcers
• Weight loss or gain
• Dry eyes
• Easy bruising
• Anxiety, depression, headaches, and memory loss

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Lupus can also lead to complications in several areas of the body. These include:

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• Kidneys - serious kidney damage is a primary cause of death for lupus sufferers.
• Central nervous system - lupus can cause headaches, dizziness, memory problems, seizures, and behavioral changes.
• Blood and vessels - lupus causes an increased risk of anemia, bleeding, blood clotting, and vessel inflammation
• Lungs - noninfectious pneumonia and difficulty breathing due to inflammation of the chest cavity are more likely with lupus
• Heart - heart muscle and artery inflammation are more likely with the disease, and lupus increases the chances of cardiovascular disease and heart attacks.
• Infection - lupus treatments tend to depress the immune system making your body more vulnerable to infection.
• Cancer - lupus increases the risk of cancer, especially of non-Hodgkin's lymphoma, lung cancer, and liver cancer
• Bone tissue death - a lower blood supply to bone tissue leads to tiny breaks and eventual death of bone. This is most common in the hip bone.
• Pregnancy - lupus increases the risk of miscarriage, hypertension during pregnancy, and preterm birth.

What is Lupus? - Video

A short video by Expert Village explaining a bit about what Lupus is.

How is lupus diagnosed?

As signs and symptoms vary considerably from person to person, there is no single diagnostic test that can confirm lupus. In addition, signs and symptoms tend to change over time and are similar to those of other disorders and diseases. These fluctuations in disease activity make lupus extremely challenging to diagnose.

Currently, doctors use guidelines established by The American College of Rheumatology (ACR) to diagnose lupus (SLE). The guidelines focus on eleven abnormalities that, when combined, suggest that the patient has lupus. To be classified as having SLE, a patient must meet 4 of the following 11 symptoms at any time since the onset of the disease:

1. Serositis - inflammation of the membrane around the lungs (pleuritis) or the heart (pericarditis)
2. Mucosal ulcers - small sores found in the lining of the mouth and nose
3. Arthritis - nonerosive arthritis (tenderness, swelling, pain) of two or more peripheral joints
4. Photosensitivity - skin rash or other symptoms caused by exposure to ultraviolet light
5. Blood disorder - hemolytic anemia (low red blood cell count), leucopenia and lymphopenia (low white blood cell count), or thrombocytopenia (low platelet count)
6. Renal (kidney) disorder - high protein count in urine
7. Antinuclear antibody test positive
8. Immunologic disorder - positives on anti-Smith, anti-ds DNA, antiphospholipid antibody tests.
9. Neurologic disorder - seizures or psychosis
10. Malar rash - rash on cheeks
11. Discoid rash - red, scaly patches on skin that cause scarring

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In addition to the above tests, doctors will often conduct a variety of blood tests such as:

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• Complete blood count (CBC) to detect anemia, low platelet count, and low white blood cell count
• Erythrocyte sedimentation rate (ESR) to determine the rate at which red blood cells settle to the bottom of a tube in an hour. Rates faster than normal may indicate lupus or another systemic disease, inflammatory condition, or infection.
• Kidney and liver assessment to look for certain enzymes and albumin
• Urinalysis to measure protein levels or red blood cells in the urine
• Syphilis test to determine if anti-phospholipid antibodies are in the blood.

How is lupus treated?

There is currently no cure for lupus, nor has there been a new drug to treat the disease in the last 50 years, although there are a number of new drugs currently being researched or in clinical trials. However, early diagnosis and proper medical treatment can significantly help control the disease and its symptoms. Treating lupus effectively consists of minimizing symptoms, reducing inflammation and pain, helping maintain normal function, and preventing serious complications.

Since the disease affects each person differently, treatments are usually tailored to the specific problems that arise in each person. Medications and dosages will also vary depending on the severity of the disease.

When lupus presents with mild or moderate symptoms, the following medications are commonly used in treatment:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin, naproxen sodium (Aleve), and ibuprofen (Advil, Motrin, others). Common side effects of NSAIDs include stomach bleeding and an increased risk of heart problems.
• Antimalarial drugs such as Hydroxychloroquine (Plaquenil). There is no known relationship between lupus and malaria, but malaria medicines have been useful in treating lupus symptoms and haven prevented flares of the disease. Side effects include vision problems and muscle weakness.
• Corticosteroids to counter inflammation. Serious long-term side effects include weight gain, easy bruising, osteoporosis, hypertension, diabetes, and increased risk of infection. The risk of osteoporosis can be reduced by taking calcium and vitamin D supplements.

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When lupus presents with severe or aggressive symptoms, the following treatments are commonly used:

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• High-dose corticosteroids. These may be taken intravenously or orally to control dangerous signs or symptoms of lupus. However, serious side effects have been observed such as infections, mood swings, hypertension and osteoporosis. Doctors tend to administer the lowest dose possible that will control symptoms, reducing the dosage over time.
• Immunosuppressive drugs such as cyclophosphamide (Cytoxan) and azathioprine (Imuran). These drugs suppress the immune system and may be helpful in serious lupus cases. They also carry a risk of serious side effects such as an increased risk of infection, liver damage, infertility and an increased risk of cancer.

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Other common treatments for specific signs and symptoms include:

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• Staying out of the sun and wearing sun block to prevent skin rashes. Indoor fluorescent lighting can also trigger skin rashes in some people with lupus. Topical corticoid steroids may be used to treat skin rashes in addition to oral steroids and antimalarial drugs.
• Medication to treat fatigue. Difficulty sleeping, depression and poorly controlled pain are all potential causes of fatigue, and doctors will treat these underlying causes. Medications such as corticosteroids and antimalarial drugs may be used if the cause of fatigue cannot be determined.
• NSAIDs, antimalarial drugs or corticosteroids to treat swelling around the heart and lungs that causes chest pain.

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In addition to medications, physicians recommend that lupus patients take good care of themselves. Patients may see a reduction in the frequency and severity of flares if they make healthy lifestyle choices such as:

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• Regular exercise.
• Becoming educated about lupus.
• Not smoking.
• Eating a healthful, balanced diet.
• Surrounding oneself with a support system of family, friends, and health professionals.

Living with lupus

Although there is no cure for lupus, there are several measures that patients can take to cope with the disease.

• Sun care - Use sunscreen with an SPF of at least 15 that can block both UVA and IVB rays.
• Diet - Eat a nutritious and well-balanced diet with limited sugar and salt intake if on corticosteroids. There is some evidence that fish has anti-inflammatory properties, but alfalfa sprouts may increase inflammation.
• Pain management - Apply moist heat to painful joints or soak in a hot tub or Jacuzzi.
• Exercise - Low-impact walking, swimming, aerobics, and bicycling may help prevent muscle atrophy and lower the risk of osteoporosis.
• Rehabilitation - Physical, occupational, and vocational therapists can help you to strengthen muscles, exercise, lower stress, recommend assistive devices, train for a job that does not exacerbate symptoms
• Don't smoke. Quit if you are a smoker.
• Climate - Changes in pressure can exacerbate symptoms. Try to live somewhere with minimal changes in climate and pressure.
• Fatigue - Control fatigue by remaining active and resting for an appropriate amount of time.
• Relationships - Maintain good relationships with the physicians that are helping you to manage lupus. Keep appointments, be honest, take medicines, and respect their time.
• Pregnancy - Keep aware and consult with a doctor about risks associated with pregnancy for you and your child.
• Cognitive function - A psychologist or cognitive therapists may be helpful if lupus leads to cognitive dysfunction or memory loss.

This is Lupus? - Video

A video from the Lupus Foundation. Individuals whoase lives have been greatly affected by lupus describe the suffering caused by disease and offer ways that you can help find a cure.

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