What Is Lupus?

Lupus is a chronic autoimmune disease that can damage any part of the body (skin, joints, and/or organs). “Chronic” means that the signs and symptoms tend to last longer than six weeks and often for many years.

In lupus, something goes wrong with the immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune systems produce proteins called “antibodies” which protect the body from these invaders.

“Autoimmunity” means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”). As a result, it creates autoantibodies that attack and destroy healthy tissue.

These autoantibodies cause inflammation, pain, and damage in various parts of the body.

Different Types of Lupus:

Systemic lupus erythematosus or SLE

Systemic lupus is the most common form of lupus—it’s what most people mean when they refer to “lupus.” Systemic lupus can be mild or severe. Below is a brief description of some of the more serious complications involving major organ systems.

Inflammation of the kidneys—called lupus nephritis—can affect the body’s ability to filter waste from the blood. It can be so damaging that dialysis or kidney transplant may be needed.

Inflammation of the nervous system and brain can cause memory problems, confusion, headaches, and strokes.

Inflammation in the brain’s blood vessels can cause high fevers, seizures, and behavioral changes.

Hardening of the arteries or coronary artery disease—the buildup of deposits on coronary artery walls—can lead to a heart attack.

Cutaneous lupus erythematosus or Discoid lupus

This form of lupus is limited to the skin. Although cutaneous lupus can cause many types of rashes and lesions (sores), the most common—called discoid rash—is raised, scaly and red, but not itchy. Areas of rash appear like disks, or circles.

Another common example of cutaneous lupus is a rash over the cheeks and across the bridge of the nose, known as the butterfly rash. Other rashes or sores may appear on the face, neck, or scalp (areas of the skin that are exposed to sunlight or fluorescent light), or in the mouth, nose, or vagina. Hair loss and changes in the pigment, or color, of the skin are also symptoms of cutaneous lupus.

Approximately 10 percent of people who have cutaneous lupus will develop systemic lupus. However, it is likely that these people already had systemic lupus, with the skin rash as their main symptom.

Drug-Induced lupus erythematosus

Drug-induced lupus is a lupus-like disease caused by certain prescription drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus, but it rarely affects major organs.

The drugs most commonly connected with drug-induced lupus include:

Hydralazine—Treatment for high blood pressure or hypertension

Procainamide—Treatment for irregular heart rhythms

Isoniazid—Treatment for tuberculosis

Drug-induced lupus is more common in men because they take these drugs more often; however, not everyone who takes these drugs will develop drug-induced lupus. Lupus-like symptoms usually disappear within six months after these medications are stopped.

Neonatal lupus

Neonatal lupus is not a true form of lupus. It is a rare condition that affects infants of women who have lupus and is caused by antibodies from the mother acting upon the infant in the womb. At birth, the infant may have a skin rash, liver problems, or low blood cell counts but these symptoms disappear completely after several months with no lasting effects. Some infants with neonatal lupus can also have a serious heart defect. With proper testing, physicians can now identify most at-risk mothers, and the infant can be treated at or before birth.

Most infants of mothers with lupus are entirely healthy.

Diagnosing Lupus

To help doctors diagnose lupus, this list of 11 common criteria, or measures, was developed by the American College of Rheumatology (ACR). ACR is a professional association of rheumatologists. Rheumatologists are the doctors who specialize in treating diseases of the joints and muscles, like lupus. If you have at least four of the criteria on the list, either at the present time or at some time in the past, there is a strong chance that you have lupus.

Malar rash – a rash over the cheeks and nose, often in the shape of a butterfly

Discoid rash – a rash that appears as red, raised, disk-shaped patches

Photosensitivity – a reaction to sun or light that causes a skin rash to appear or get worse

Oral ulcers – sores appearing in the mouth

Arthritis – joint pain and swelling of two or more joints in which the bones around the joints do not become destroyed

Serositis – inflammation of the lining around the lungs (pleuritis) or inflammation of the lining around the heart that causes chest pain which is worse with deep breathing (pericarditis)

Kidney disorder – persistent protein or cellular casts in the urine

Neurological disorder – seizures or psychosis

Blood disorder – anemia (low red blood cell count), leukopenia (low white blood cell count), lymphopenia (low level of specific white blood cells), or thrombocytopenia (low platelet count)

Immunologic disorder – anti-DNA or anti-Sm or positive antiphospholipid antibodies

Abnormal antinuclear antibody (ANA)

Reducing Time to Diagnosis

 Lupus is a disease that is known for being difficult to diagnose because the symptoms are different from person to person, they mimic the symptoms of many other diseases, and they can come and go. It can sometimes take several years to receive an official diagnosis. To diagnose lupus as early as possible, there are three important things you can do:

Educate yourself about lupus. Learn as much as you can about the signs and symptoms of lupus. Our “I Might Have Lupus” section is a great place to look  for answers to frequently asked questions about diagnosis.

Communicate with your doctor. Tell him or her about any symptoms you might be experiencing and any family history of lupus or other autoimmune diseases. Try keeping track of your symptoms so that your doctor can see how they change over time.

See a rheumatologist. If you have learned about lupus and talked to your primary care doctor, and you still think lupus is a possibility, make an appointment to see a rheumatologist. He or she can help determine whether you have lupus.

Doctors Who Treat Lupus

A diagnosis of lupus may be made by any physician (family practitioner, Internist, or pediatrician) if multiple symptoms and laboratory test results point toward the disease. However, if symptoms develop gradually over time, as is often the case with lupus, the diagnosis may not be as obvious and there may be visits to multiple physicians before a diagnosis can be confirmed.

The form of lupus and its symptoms determine what type of doctor you will see for treatment. Most people with mild to moderate disease will see a rheumatologist (or pediatric rheumatologist if a child or adolescent has lupus), a physician who specializes in diseases of joints and muscles.

Since lupus can cause damage to any part of the body, other specialists may be necessary such as a:

Dermatologist – a doctor who specializes in diseases of the skin such as cutaneous lupus

Cardiologist – a doctor who specializes in diseases of the heart

Nephrologist – a doctor who specializes in diseases of the kidney

Neurologist – a doctor who specializes in diseases of the brain and nervous system

Gastroenterologist – a doctor who specializes in diseases of the gastrointestinal tract

Pulmonologist – a doctor who specializes in diseases of the lungs

Perinatologist – a specialized obstetrician/gynecologist who focuses on complicated and high-risk pregnancies

Lupus Facts You Should Know:

  • Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
  • Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above. However, some treatments for lupus may include immunosuppressant drugs that are also used in chemotherapy.
  • Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
  • Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
  • More than 16,000 new cases of lupus are reported annually across the country.
  • Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
  • It is believed that 5 million people throughout the world have a form of lupus.
  • Lupus strikes mostly women of childbearing age. However, men, children, and teenagers develop lupus, too. Most people with lupus develop the disease between the ages of 15-44.
  • Women of color are two to three times more likely to develop lupus than Caucasians.
  • People of all races and ethnic groups can develop lupus.

Treatments:

Treatment for lupus depends on your signs and symptoms. Determining whether your signs and symptoms should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor.

As your signs and symptoms flare and subside, you and your doctor may find that you’ll need to change medications or dosages. The medications most commonly used to control lupus include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs).Over-the-counter NSAIDs, such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be used to treat pain, swelling and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects of NSAIDs include stomach bleeding, kidney problems and an increased risk of heart problems.
  • Antimalarial drugs.Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), affect the immune system and can help decrease the risk of lupus flares. Side effects can include stomach upset and, very rarely, damage to the retina of the eye. Regular eye exams are recommended when taking these medications.
  • Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (A-Methapred, Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.
  • Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate mofetil (CellCept) and methotrexate (Trexall). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
  • A different type of medication, belimumab (Benlysta) administered intravenously, also reduces lupus symptoms in some people. Side effects include nausea, diarrhea and infections. Rarely, worsening of depression can occur.
  • Rituximab (Rituxan) can be beneficial in cases of resistant lupus. Side effects include allergic reaction to the intravenous infusion and infections.

Cytoxan Chemotherapy Your doctor may have prescribed you the immunosuppressant drug Cytoxan (cyclophosphamide) if your lupus has gotten severe. The “gold standard” drug regimen for treating severe lupus is Cytoxan in combination with the corticosteroid methylprednisolone.

Cytoxan is actually a cancer drug, but in lupus patients, it’s used to treat serious kidney inflammation (including lupus nephritis) or other complications that threaten the organs.

Immunosuppressives such as Cytoxan are used in the treatment of lupus for two main reasons:

  • They are potent drugs that help control disease activity in major organs.
  • They may reduce or eliminate the need for steroids.

Cytoxan is usually only given for three to six months until a patient goes into lupus remission.