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Lupus

What is lupus?

Lupusmore technically known as lupus erythematosusis a systemic autoimmune disease. In other words, it’s a disease that happens when a person’s immune system starts a misguided attack through the body. It affects roughly 1.5 million Americans, mostly younger women. It strikes about nine times more women than men, and about 80 percent of these women are between the ages of 15 and 45. However, it can develop in anyone, at any age.

Unfortunately, there’s no cure for lupus, but the right combination of medications may control your immune system. There has been great progress in recent decades. As recently as 1965, only half of all lupus patients survived longer than 10 years; today more than 90 percent make it past 10 years.

How does lupus get its start?

There are still a lot of unanswered questions about lupus, but researchers have definitely been making progress. Scientists believe that certain mutations in genes can raise the odds of the disease. Lupus is more common among some ethnic groups, including Asians, Latinos, and African Americans. It also runs in families, but genetics is not the only factor involved — something beyond genes seems to send the disease into action. Studies show that if one identical twin has lupus, the other twin has only a 26 to 70 percent chance of having it, a clear sign that it takes more than genes to get the disease.

No one is sure what triggers lupus, but the list of suspects currently includes ultraviolet light, environmental chemicals, certain pharmaceutical drugs, and viruses or other infectious agents. Female hormones can make the immune system more reactive, which could explain why the majority of people with lupus are women in their fertile years. Researchers think the full picture is very complicated, and think that a combination of these factors may trigger the disease.

How is lupus diagnosed?

Lupus appears in different ways in different people, so it can be difficult to recognize. Most commonly, a person with lupus will show various symptoms (often a combination of rashes, fatigue, painful or swollen joints, low-grade fever, loss of appetite, and weight gain or loss) for about three years before the diagnosis is made. They’ve often visited three to five doctors by this time.

If your primary care doctor thinks you may have lupus, you’ll probably get a referral to a rheumatologist or an immunologist for a full evaluation. At your visit, the specialist will ask you about your personal and family health history, do a physical exam covering all of the body’s major systems, and order some lab tests. According to the American College of Rheumatology, a person with at least four of the following 11 symptoms may be diagnosed with systemic lupus erythematosus:

On the Skin

1. Butterfly-shaped rash on cheeks and nose (this resembles a wolf’s facial markings, another explanation for the name of the disease)

2. Thick disk-shaped rash (usually on areas that have been exposed to the sun)

3. Sensitivity to the sun (exposure to the sun’s ultraviolet light triggers a rash)

4. Recurrent sores in the mouth or nose

In Organs or Joints

5. Arthritis (tenderness, swelling, or fluid buildup in at least two joints)

6. Serositis (inflammation of lining around the heart or lung)

7. Kidney disorder (signaled by protein or other abnormal material in the urine)

8. Neurologic symptoms (seizures or psychosis with no other explanation)

In the Blood (Revealed by Lab Tests)

9. Positive antinuclear antibody (ANA) test (this means that the body is making antibodies to the DNA-containing cores of its own cells)

10. Blood abnormalities (hemolitic anemia, which destroys certain red blood cells; or an increase in certain white blood cells)

11. Immunologic disorders (these may include the presence of certain immune cells called lupus erythamatosus (LE) cells, false-positive tests for syphilis, or “anti-Sm antibodies,” named after a certain Mrs. Smith, which are antibodies to a person’s own DNA and interfere with RNA. Although anti-Sm antibodies are seen in about 30 percent of lupus patients, a patient may have the disease even if the antibodies aren’t found.)

Diagnosis is so tricky because there isn’t one single test for lupus — it’s a subjective call, based on many tests and symptoms. For example, about 97 percent of people with lupus have a positive ANA test, but of all the people who test positive, only 10 percent have lupus. Also, lupus has many symptoms in common with other autoimmune disorders including rheumatoid arthritis and fibromyalgia.

Are there different kinds of lupus?

When people say “lupus,” they usually mean systemic lupus, or SLE. But doctors recognize four “versions” of the disease:

  • DLE, or discoid lupus erythematosus, affects about 10 percent of lupus patients. In this type, the immune reactions are limited to the skin.
  • SLE, or systemic lupus erythematosus, is most common type, affecting about 70 percent of people with lupus. The disease has system-wide effects. It has two sub-types, which occur in roughly equal numbers:
  • Non-organ threatening SLE, in which the vital organs are not affected even though the disease is system wide. People with this type have normal life spans.
  • Organ-threatening SLE, in which the immune system may attack the kidneys, lungs, heart, liver, or brain. This type can be life-threatening and needs to be controlled with steroids. Steroids themselves generally have serious side effects.
  • DILE, or drug-induced lupus erythematosus, develops as a result of a side effect of certain drugs on the body and accounts for about 10 percent of total cases. About half the time, drug-induced cases meet the criteria for SLE. This kind of lupus affects men and women equally, is generally less severe, and goes away after the person stops taking the particular drug, but may take months or even years to completely resolve. Dozens of drugs have been proven to cause this type of lupus. These include hydralazine (Apresoline) and methyldopa (Aldomet), both used for hypertension; quinidine and procainamide (Pronestyl), used to treat irregular or rapid heartbeats; and chlorpromazine (Thorazine), used to treat several psychological problems including schizophrenia. Many of these drugs are being used less frequently because of their side effects.
  • Crossover or overlap syndrome and mixed connective tissue disorder accounts for the remaining 5 to 10 percent of patients who meet the criteria for lupus. According to Daniel J. Wallace, MD, these people also fit the profile for another autoimmune disorder such as scleroderma or rheumatoid arthritis. If they have a particular antibody (anti-RNP), doctors call the condition mixed connective tissue disorder (MCTD); otherwise they call it crossover or overlap syndrome.

What is the treatment for lupus?

The treatment plan will depend on how severe your symptoms are, and which organs are affected. As is true for other autoimmune diseases, there isn’t an elegant treatment. Your doctor may prescribe one or some combination of the following:

  • Anti-inflammatories. These drugs lower fever, ease headache, reduce joint and muscle pain, and relieve serositis (inflammation of the lung and heart linings). Nonsteroidal anti-inflammatories (NSAIDs) include common over-the-counter drugs such as aspirin and ibuprofen (Advil) as well as the newer drugs recently approved for arthritis (like Celebrex).
  • Antimalarials. Drugs used to treat malaria, such as chloroquine (Aralen) and hydroxychloroquine (Plaquenil), act as mild stimulants, reduce inflammation, block ultraviolet light so it doesnt damage skin, lower bloodstream cholesterol, and change the acidity in cells (which makes it harder for the body to produce antibodies to the cells DNA).
  • Steroids. These drugs are commonly used when symptoms dont respond to NSAIDs or antimalarials, or when the lupus is the organ-threatening type. Your body naturally produces these hormones, but in high doses they interfere with the inflammation and joint pain triggered by an overactive immune system. Prednisone is the corticosteroid most often prescribed for lupus. Steroid ointments such as hydrocortisone are useful for treating skin rashes.
  • Chemotherapy drugs (immunosuppressives). This class of drugs is used to treat serious system-wide cases of lupus in which major organs are affected. Theyre also useful in cases of severe muscle inflammation or stubborn arthritis. The drugs most frequently used for lupus are azathioprine (Imuran), cyclophosphamide (Cytoxan), and methotrexate (Rheumatrex).

Are there any alternative therapies that work for lupus?

There are no proven alternative ways to treat lupus, although many complementary therapies can give your immunity a boost and lower your stress level. Mind-body techniques such as yoga, meditation, hypnotherapy, and guided imagery may help you relax and feel more positive. Acupuncture helps some people cope with joint and muscle pain. Fish oil may have anti-inflammatory properties, and some doctors recommend eating fish several times a week. If your lupus is mild, it may be worth looking into natural anti-inflammatory supplements such as fish oil capsules, black currant oil or grapeseed oil, ginger, feverfew, and turmeric. (These are not proven remedies, however.) It’s important to consult with your doctor before trying any of these supplements because they might interact with your medications.

What else can I do to stay healthy?

If you pay attention to your body’s signals, you can learn to recognize the signs of an approaching flare-up. Here are a few simple ways to gain more control over your condition:

  • Eat well. Regular, balanced meals keep your energy up. Doctors often recommend cutting back on salt, eating calcium-rich foods, and having fish regularly — as previously mentioned, the fatty acids in fish may decrease inflammation. Experiment and see what works for you. Whatever you do, avoid alfalfa sprouts — they contain canavanine, a natural toxin that harms the immune system and leads to flare-ups.
  • Don’t smoke. Smoking can worsen the effects of lupus on your heart and blood vessels. If you smoke, quit.
  • Get lots of sleep, and take steps to reduce the stress in your life. When you’re stressed out, your immune system suffers. Mind-body healing methods help some people feel more relaxed, positive, and in control of their lives.
  • Stay active. Even if you feel tired or achy, try to take a walk or bike ride every day. Also, doing some strength-building exercises can keep your muscles and bones from weakening.
  • Stay out of the sun. Ultraviolet rays can trigger an immune response in people with lupus. Every time you go out, you should not only wear sunscreen but cover up your skin with long sleeves and a hat.

Will I ever recover?

With the right lifestyle and medication, most people can get their condition under control and live normal, active lives. If your lupus is relatively mild, you’ll live about as long as someone without lupus. Even if you have an active, organ-threatening case, you’re likely to live a long time once you make it past the critical first two to three years. Unfortunately, taking high doses of steroids for 10 to 15 years can take a toll on your body and cause complications such as osteoporosis and fractures, thick skin, cataracts, easy bruising, and diabetes. That’s why medical researchers are striving to develop more targeted, less toxic drugs for lupus.

References

Lupus Foundation of America. Introduction to Lupus. 2007. http://www.lupus.org

Foundation of America. Laboratory Tests Used in the Diagnosis of Lupus.

Lupus Foundation of America. Drug-Induced Lupus.

Mayo Clinic. Lupus. 2009. http://www.mayoclinic.com/health/lupus

Lupus.National Institute of Arthritis and Musculoskeletal and Skin Disease. 2009. http://health.nih.gov/topic/Lupus

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