Lupus
“MARK OF THE WOLF”

Lupus erythematosus (pronounced loo-pus air-re-them-atoe-sus) is an autoimmune disorder like RA. The disease was named by clinicians who observed that the skin problems that often signal the condition resembled the facial markings of a wolf (lupus means wolf; erythematosus means redness). The cause of lupus is generally unknown.

Researchers theorize that the most likely culprit is a genetic disposition toward the disease, combined with a subsequent exposure to some environmental insult or infection that leads to a “confused” immune system that attacks the body’s own tissues. Up to 5 percent of sisters and daughters of patients with lupus may also develop the disease. It is not uncommon for relatives of lupus patients to have abnormal antibodies in their blood, but with no symptoms of the disease. Lower levels of anti-nuclear antibodies (ANA) are often found when RA is also present.

It used to be that only the most severe cases of lupus were diagnosed. Now, due to the sensitivity of newer ANA blood tests, milder cases of lupus are diagnosed more quickly. Most people with lupus live a normal life with few changes in lifestyle. Nevertheless, detecting the condition early allows patients to be monitored for evidence of more serious illness, and treated appropriately.

Treatment of lupus is based on the underlying symptoms. Plaquenil® (hydroxychloroquine sulfate), which is also used in RA, may help to control the skin and joint symptoms of lupus, as well as the fatigue. When internal organs such as the kidneys, heart or lungs are involved, stronger medications may be prescribed. These include Imuran® (azathioprine), CellCept® (mycophenolate mofetil) or Cytoxan® (cyclophosphamide). These medications may be very effective, but they can pose an increased risk of potential side effects. Some people with lupus do not have to take medications regularly. Prescription drugs (such as corticosteroids) may be ordered as needed for flare-up of symptoms.

Four Types of Lupus

There are four types of lupus. All feature the tell-tale skin rash that is the hallmark of the disorder. None of the four types of lupus are infectious. Nor are they a type of cancer or malignancy. Like RA, people with lupus have an overactive immune system. The number of cases of lupus in the United States is unknown, but experts estimate that up to 1.5 million people may be affected with the disease. Ninety percent of lupus patients are women.

Drug-induced lupus. Drug-induced lupus is a rare condition caused from long-term exposure to certain medications. The condition clears up once the offending medication is stopped. However, the presence of anti-nuclear antibodies (ANA), a marker for lupus, may continue to show in blood tests for a year or more.

Discoid lupus. Discoid lupus is identified by a skin rash with raised, red, scaling areas. These lesions sometimes leave scars, and are typically seen on the face, scalp and other sunexposed areas. Most people with discoid lupus do not have internal organ involvement, as is seen with the systemic form of the disease.

Subacute lupus. Like discoid lupus, subacute lupus is also associated with a skin rash with raised, red scaly patches. However, unlike discoid lupus, this form of the disease does not scar.

Systemic lupus erythematosus (SLE). In the 1890s, the famed physician Sir William Osler observed that internal organs — or systems — can also be involved in addition to skin changes associated with lupus. Thus, the term systemic lupus erythematosus (SLE) was coined. Symptoms of SLE include arthritis, rash, and flu-like symptoms such as aching joints and muscles and fatigue. Infection and sunlight may trigger lupus, but symptoms seem to come and go for no apparent reason. This makes the condition harder to diagnose.

SLE commonly affects the heart or lungs, where there is usually an inflammation of the organ’s lining. This may cause chest pain, especially with breathing. The kidneys may also be involved in SLE. Patients may have no symptoms, but a urine test can detect evidence of inflammation. Other systems and regions affected by SLE may include the bone marrow (blood cells), the brain and blood vessels.

Lupus Isn’t Always a Clear-Cut Diagnosis

Lupus can be difficult to diagnose. The American College of Rheumatology established 11 criteria to help identify the disorder. A person with lupus usually has four or more of the following symptoms:

1. Malar “butterfly” rash on the cheeks.

2. Discoid skin lesions.

3. Sun sensitivity, where a rash develops from exposure to sun or UV light.

4. Mouth sores, usually on the roof or back of the mouth (typically not painful).

5. Arthritis, with prolonged morning stiffness, usually up to an hour, improving as the day goes on.

6. Abnormal urine test showing large amounts of protein.

7. A history of seizures or psychiatric problems.

8. Sharp pain during breathing due to inflammation of the lining of the lungs or heart, which worsens with deep inhalations (pleurisy).

9. Low white blood count, low platelet count or evidence of anemia.

10. The presence of antibodies to double-strand DNA (ds-DNA), or of Smith (Sm) antibodies which are specific for diagnosing lupus.

11. A positive anti-nuclear antibody (ANA) test. 98 percent of people with lupus have this antibody.

Scott Zashin, MD, PA is a respected Texas lupus Doctor/Specialist with offices in Dallas & Plano. The above information about lupus is from Dr. Zashin's arthritis book: Arthritis Without Pain, a comprehensive guide for patients considering or undergoing treatment with the TNF blockers Enbrel®, Remicade®, or Humira®. All rights reserved.