Lupus is an autoimmune disease that causes the immune system to mistakenly work against the body's own tissues. It can be hard to diagnose because it can affect almost any organ in the body, and its symptoms — including joint pain, fatigue, muscle pain, rash, mouth ulcers, and hair loss — vary widely from patient to patient. That is one of the greatest challenges of treating the disease for both doctors and their patients.
A healthy immune system produces proteins called antibodies that normally protect the body against bacteria and viruses that cause infections. But when someone has lupus, the immune system can't tell the difference between the body's healthy cells and bacteria and viruses, so the antibodies attack the body's healthy cells.
Lupus affects 1.5 million people in the United States, including an estimated 10,000 children. Nearly 90% of adults diagnosed with lupus are female, although the male to female ratio is much less different for kids and teens.
What Causes Lupus?
Although the cause of lupus is unknown, researchers think that many factors may trigger the disease.
Genetics might play a role. Some people may have a genetic predisposition to lupus that is then activated by an infection, certain medications, or extreme physical or emotional stress. The hormone estrogen also may play a role and could help explain why it's more common in females than males.
Lupus also happens more often in African Americans, Asian Americans, Latinos, and Native Americans than in caucasians.
Types of Lupus
The three main types of lupus are:
- Systemic lupus erythematosus, or SLE, which can affect multiple organs in the body and is the most common form of the disease. Although SLE typically develops in people in their twenties, thirties, or forties, some people first notice symptoms during childhood or adolescence (after age 10).
SLE can be difficult to diagnose because no two patients will have an identical set of complaints, and the symptoms can sometimes be mistaken for those of other conditions, like juvenile idiopathic arthritis, Crohn's disease, Lyme disease, and mononucleosis.
- Cutaneous (or skin) lupus, which usually affects only the skin and is characterized by a rash on the scalp, legs, or arms. The rash has sharply defined patches and can be raised from the skin.
There are two types of cutaneous lupus: discoid lupus erythematosus (DLE), which can cause scarring; and subacute cutaneous lupus erythematosus (SCLE), which doesn't cause scars. Discoid lupus affects approximately 15% of all lupus patients, but is rare in children. Occasionally, discoid lupus occurs with systemic lupus.
- Drug-induced lupus, which accounts for about 10% of all lupus cases, occurs as a reaction to specific kinds of medications. In kids, anti-seizure medications, thyroid medications, and acne medications can cause lupus. The symptoms are similar to those of the systemic form, but are usually milder and go away after the medication is stopped.
The two rashes commonly associated with lupus are:
- malar rash — a rash across the cheeks and the bridge of the nose (also called a "butterfly" rash because it is shaped like a butterfly)
- discoid rash — circular, red or hyperpigmented raised patches of skin that often occur on the face, neck, or chest
Other common symptoms:
- sensitivity to ultraviolet light — either sunlight or, occasionally, certain types of fluorescent light (also called photosensitivity) — that results in a rash
- ulcers in the nose or mouth
- nonerosive arthritis (arthritis that does not destroy the bones around the joints)
- inflammation of the lining around the heart, abdomen, or lungs (called serositis)
- kidney problems, either mild (with no early symptoms) or severe (such as those that lead to swelling in the legs)
- neurological disorders, such as seizures or psychosis
- blood problems, such as a low red blood cell count (anemia), a low white blood cell count (leukopenia), or a low platelet count (thrombocytopenia)
- problems with the immune system
- positive blood test for antinuclear antibodies (ANA), specific proteins that are a hallmark of rheumatic or autoimmune disease. More than 95% of lupus patients have a positive ANA.
Someone with four or more of these signs or symptoms is likely to have SLE. Most patients don't develop all of them. Before making a diagnosis, doctors perform a physical exam and take blood tests to rule out any other diseases.
In addition to those symptoms, patients with lupus often have fever, weakness, fatigue, or weight loss. They may experience muscle aches, loss of appetite, swollen glands, hair loss, or abdominal pain, which can be accompanied by nausea, diarrhea, and vomiting.
Sometimes the fingers, toes, nose, or ears will be particularly sensitive to cold and will turn blue and white in cold temperatures, a condition known as Raynaud's phenomenon.
There is no known cure for lupus, but the symptoms of the disease can be controlled. Often a patient with lupus has a health care team that includes specialists who can help treat the symptoms. That team may include:
- a rheumatologist (a doctor who specializes in conditions of the joints and connective tissues)
- a nephrologist (a doctor who specializes in kidney diseases)
- an advanced practice nurse
- a social worker
- other consulting doctors, like a dermatologist, psychologist, or infectious disease specialist
Part of managing lupus is preventing flares — times when the disease gets worse. During a flare-up, a person with lupus may feel much more tired, sick, feverish, and achy than usual. Almost all lupus patients take medication to control inflammation and reduce the risk of flares.
Doctors often prescribe corticosteroids to control inflammation. These aren't the same steroids some athletes take. If a doctor prescribes one of these medicines, the dosage and any side effects will be carefully monitored.
For day-to-day muscle and joint pain, patients can take acetaminophen or any of a variety of nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen, or naproxen.
Almost all patients with lupus take antimalarial drugs (medicines first developed to prevent and treat malaria that also have proved helpful with lupus). Antimalarial drugs often help treat skin rashes and joint pain, and help prevent coronary artery disease and the involvement of other organ systems in lupus.
Some children who also have kidney disease, very low blood cell counts, or other organ involvement, may require more aggressive treatment with immunosuppressive drugs, which lower the body's immune system responses.
In 2011, belimumab was approved by the U.S. Food and Drug Administration (FDA) for the treatment of lupus, especially for arthritis, rashes, and fatigue.
Making Lifestyle Changes
Though the course that lupus takes cannot be predicted, certain lifestyle changes may help minimize flare-ups. Patients should avoid too much sun exposure by wearing sunscreen and protective clothing when outside.
Regular exercise can help prevent fatigue and joint stiffness. A balanced diet and sufficient rest also are important for maintaining general health and well-being.
When to Call the Doctor
Kids and teens with lupus are prone to the usual childhood illnesses, such as a viral infection or diarrhea. However, a fever, rash, or mouth sore might indicate the beginning of a flare. As you and your child become more familiar with the disease, you may learn to recognize signs that a flare-up is around the corner.
Call the doctor immediately if any of these symptoms appear:
- bloody stools
- easy bruising (with or without nosebleeds)
- chest or abdominal pain
- new or high fever
The Outlook for Lupus
The outlook for lupus patients is constantly improving. Over the past few decades, better tools to diagnose and treat lupus have remarkably improved the lives of those who live with the disease.
Reviewed by: AnneMarie C. Brescia, MD
Date reviewed: July 2014