Causes, Diagnosis, and Treatment of Lupus

Each person with lupus has slightly different symptoms that can range from mild to severe and may come and go over time. A red skin rash—the so-called butterfly or malar rash—may appear across the nose and cheeks. Rashes may also occur on the face and ears, upper arms, shoulders, chest, and hands and other areas exposed to the sun. Because many people with lupus are sensitive to sunlight, skin rashes often first develop or worsen after sun exposure.

New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at different times. In some people with lupus, only one system of the body, such as the skin or joints, is affected. Other people experience symptoms in many parts of their body. Just how seriously a body system is affected varies from person to person.

Causes

Lupus is a complex disease, and its cause is not fully understood. Although there are several kinds of lupus, systemic lupus erythematosus (SLE) is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life, as well.

Research suggests that genetics plays an important role in developing lupus. Other factors also play a role. Some of the factors scientists are studying include sunlight, stress, hormones, cigarette smoke, certain drugs, and infectious agents, such as viruses. Studies have confirmed that one virus, Epstein-Barr virus (EBV), which causes mononucleosis, is a cause of lupus in genetically susceptible people.

In lupus, the body's immune system does not work as it should. A healthy immune system produces proteins called antibodies and specific cells called lymphocytes that help fight and destroy viruses, bacteria, and other foreign substances that invade the body. In lupus, the immune system produces antibodies against the body's healthy cells and tissues. These antibodies, called autoantibodies, contribute to the inflammation of various parts of the body and can cause damage to organs and tissues.

Diagnosis

Diagnosing lupus can be difficult. It may take months or even years for doctors to piece together the symptoms to diagnose this complex disease accurately. Making a correct diagnosis of lupus requires knowledge and awareness on the part of the doctor and good communication on the part of the patient.

Treatment

Most people will see a rheumatologist for their lupus treatment. A rheumatologist is a doctor who specializes in rheumatic diseases (arthritis and other inflammatory disorders, often involving the immune system). Clinical immunologists (doctors specializing in immune system disorders) may also treat people with lupus. As treatment progresses, other professionals often help. These may include nurses, psychologists, social workers, nephrologists (doctors who treat kidney disease), cardiologists (doctors specializing in the heart and blood vessels), hematologists (doctors specializing in blood disorders), endocrinologists (doctors specializing in problems related to the glands and hormones), dermatologists (doctors who treat skin disease), and neurologists (doctors specializing in disorders of the nervous system). It is also important for people with lupus to have a primary care doctor—usually a family physician or internist (internal medicine specialist)—who can coordinate care between their different health providers and treat other problems as they arise.

The range and effectiveness of treatments for lupus have increased dramatically in recent decades, giving doctors more choices in how to manage the disease. Medications used in the treatment of lupus include the following:

• NSAIDs: For people with joint or chest pain or fever, drugs that decrease inflammation, called nonsteroidal anti-inflammatory drugs (NSAIDs), are often used.
• Antimalarials: These drugs were originally used to treat malaria, but doctors have found that they also are useful for lupus.
• Corticosteroids: Corticosteroids, such as prednisone, hydrocortisone, methylprednisolone, and dexamethasone, are related to cortisol, which is a natural anti-inflammatory hormone.
• Immunosuppressives: Immunosuppressives, such as cyclophosphamide and mycophenolate mofetil, restrain the overactive immune system by blocking the production of immune cells.
• Biologics: Belimumab, a type of agent referred to as a B-lymphocyte stimulator (BLyS) protein inhibitor, was approved by the U.S. Food and Drug Administration (FDA) in March 2011 for patients with lupus who are receiving other standard therapies, including those listed above.

There are other, less common, therapies that are used, depending on the symptoms and parts of the body affected.