Systemic Lupus Erythematosus (Lupus)

What is lupus?

Systemic lupus erythematosus, also known as SLE, or simply lupus, is a disease that is characterized by periodic episodes of inflammation of and damage to the joints, tendons, other connective tissues, and organs, including the heart, lungs, blood vessels, brain, kidneys, and skin. The heart, lungs, kidneys, and brain are the organs most affected. Lupus affects each individual differently and the effects of the illness range from mild to severe. Lupus can potentially be fatal.

The majority of people who have lupus are young women (late teens to 45). This may be due to the fact that estrogen (a female hormone) seems to be associated with lupus. Lupus affects more African-Americans, Asian Americans, Latinos, and American Indians than white Americans. Lupus in children occurs most often at the age of 15 and older. According to the Arthritis Foundation, about 25,000 children and adolescents have lupus or a related disorder.

The disease is known to have periods of flare-ups and periods of remission (partial or complete lack of symptoms). Children with lupus can have a large degree of kidney involvement. The severity of the kidney involvement can alter the survival rate of patients with lupus. In some cases, kidney damage is so severe it leads to kidney failure.

What causes lupus?

Lupus is an autoimmune disorder, which means the body's immune system attacks its own healthy cells and tissues.

Lupus is considered to be a multifactorial condition. Multifactorial inheritance means that many factors are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Multifactorial traits do recur in families because they are partly caused by genes.

A group of genes on chromosome 6 codes for the HLA (human leukocyte antigens) antigens which play a major role in susceptibility and resistance to disease. Specific HLA antigens influence the development of many common disorders, many that are autoimmune related and are inherited as multifactorial traits. When a person has the specific HLA antigen type associated with the disease, they may have a genetic susceptibility to have the condition and be more apt to develop it. The HLA antigen associated with lupus is called DR2 and DR3. It is important to understand that a person without these antigens may also develop lupus, so that HLA antigen testing is not diagnostic or accurate for prediction of the condition.

What is the immune system?

The purpose of the immune system is to keep infectious microorganisms, such as certain bacteria, viruses, and fungi, out of the body, and to destroy any infectious microorganisms that do invade the body. The immune system is made up of a complex and vital network of cells and organs that protect the body from infection.

When the immune system does not function properly, a number of diseases can occur. Allergies and hypersensitivity to certain substances are considered immune system disorders. In addition, the immune system plays a role in the rejection process of transplanted organs or tissue. Other examples of immune disorders include the following:

  • Autoimmune diseases, such as juvenile diabetes, rheumatoid arthritis, and anemia

  • Immunodeficiency diseases, such as AIDS and severe combined immunodeficiency (SCID)

Illustration of the anatomy of the immune system, adult

What are the symptoms of lupus?

Lupus symptoms are usually chronic and relapsing. The following are the most common symptoms of lupus. However, each individual may experience symptoms differently. Symptoms may include:

  • Malar rash. A rash shaped like a butterfly that is usually found on the bridge of the nose and the cheeks.

  • Discoid rash. A raised rash found on the head, arms, chest, or back.

  • Fever

  • Inflammation of the joints

  • Painful joints

  • Sunlight sensitivity

  • Hair loss

  • Mouth ulcers (sores)

  • Fluid around the lungs, heart, or other organs

  • Kidney problems

  • Low white blood cell or low platelet count

  • Raynaud's phenomenon. A condition in which the blood vessels of the fingers and toes go into spasm when triggered by factors such as cold, stress, or illness.

  • Weight loss

  • Nerve or brain dysfunction

  • Anemia

  • Decreased appetite

  • Low energy 

  • Swollen glands 

The symptoms of lupus may resemble other medical conditions or problems. Always consult your doctor for a diagnosis.

How is lupus diagnosed?

Lupus is difficult to diagnose because of the vagueness of the symptoms each person might have. There is no single test that can diagnose lupus. A diagnosis is usually confirmed based on a complete medical history, reported symptoms, and a physical examination that may include the following:

  • Blood test. This test is done to detect for certain antibodies that are present in most people with lupus.

  • Blood and urine tests. These tests are done to assess kidney function.

  • Complement test. This test is done to measure the level of complement, a group of proteins in the blood that help destroy foreign substances; low levels of complement in the blood are often associated with lupus.

  • X-rays. A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

  • Erythrocyte sedimentation rate (also called ESR or sed rate). A measurement of how quickly red blood cells fall to the bottom of a test tube. When swelling and inflammation are present, the blood's proteins clump together and become heavier than normal. Thus, when measured, they fall and settle faster at the bottom of the test tube. Generally, the faster the blood cells fall, the more severe the inflammation.

  • C-reactive protein (CRP). A protein that is elevated when inflammation is found in the body. Although ESR and CRP reflect similar degrees of inflammation, sometimes one will be elevated when the other is not. This test may be repeated to test your response to medication.

Further, the American College of Rheumatology created a set of criteria to assist doctors in making a diagnosis of lupus. The individual must have four of the 11 specific criteria to be diagnosed with lupus. It is important to remember that having some of the following symptoms does not mean that lupus is the diagnosis. The criteria include the following:

  • Malar rash. A rash shaped like a butterfly that is usually found of the bridge of the nose and the cheeks.

  • Discoid rash. A raised rash usually found on the head, arms, chest, or back.

  • Sunlight sensitivity

  • Mouth ulcers

  • Inflammation of the joints

  • Heart or lung involvement

  • Kidney problems

  • Seizures or other neurological problems

  • Positive blood tests

  • Changes in normal blood values

Treatment for lupus

There is no cure for lupus. Specific treatment for lupus will be determined by your doctor based on:

  • Your age, overall health, and medical history

  • Extent of the condition

  • Your tolerance for specific medications, procedures, and therapies

  • Expectation for the course of the disease

  • Specific organs that are affected

  • Your opinion or preference

If lupus symptoms are mild, treatment may not be necessary, other than possibly nonsteroidal anti-inflammatory medications (NSAIDs) for joint pain. Other treatment may include:

  • Hydroxychloroquine, quinacrine, chloroquine, or a combination of these medications

  • Corticosteroids (to control inflammation)

  • Immunosuppressive medication (to suppress the body's autoimmune system)

  • Monoclonal antibodies, such as belimumab and rituximab, for selected patients, depending on disease activity and the results of certain blood tests. Patients respond differently to these treatments and are carefully selected by their doctor. 

  • Liberal use of sunscreen, decreased time outdoors between 10 a.m. and 4 p.m., and wearing hats and long sleeves when outdoors, as about one-third of persons with lupus have the tendency to develop a rash in the sun

  • Rest, including at least eight to 10 hours of sleep at night; naps and breaks during the day

  • Stress reduction

  • Well-balanced diet

  • Immediate treatment of infections

Children with lupus should not receive immunizations with live viruses, including chickenpox, MMR (measles, mumps, rubella), and oral polio vaccines. Consult your child's doctor regarding all vaccines.