Systemic lupus erythematosus (SLE), commonly known as lupus, is an autoimmune illness that affects all parts of the body. The term "lupus" is also used for other, related illnesses described below.
In SLE, the immune system, which normally protects the body from foreign invaders and infection, malfunctions and instead attacks a person's own healthy body tissues. Why it does so is unknown, but most scientists believe that genetics, combined with outside triggers (such as infections, medications or other environmental factors), lead people to develop lupus. Lupus is a lifelong condition, but symptoms tend to cycle in alternate periods of "flares" (or "flares-ups") and remissions. Lupus affects women much more than men. There is no known cure, but numerous treatments are available.
If your primary care doctor suspects you may have lupus, contact a rheumatologist to confirm the diagnosis and get appropriate treatment. HSS is top ranked for rheumatology by U.S.News & World Report.
There are five recognized forms of lupus:
Symptoms vary from person to person, but the typical lupus patient is a young woman who develops arthritis of the fingers, wrists or other small joints, hair loss, a rash usually on her face, and sometimes fever, swollen lymph nodes (glands), chest pain, and/or protein in the urine. Oftentimes the first indication is an abnormal blood test that may or may not lead to illness. Symptoms usually begin in only one or two areas of the body, but more may develop over time. The most common signs and symptoms of lupus are:
Patients with lupus also characteristically have the following abnormal lab results:
In addition to discussing these and other symptoms with you, your doctor will order a variety of tests to confirm a diagnosis of lupus. These may include blood, urine and biochemical tests. People with lupus sometimes have false-positive test results for syphilis, which has nothing to do with the disease syphilis, but is an artifact of having antiphospholipid antibody.
The severity of lupus varies from mild to life threatening. Blood count abnormalities often do not need treatment. Rashes, fatigue, arthritis, and other symptoms usually respond to various forms of treatment. Kidney problems and neurologic complications are more dangerous and often require very aggressive treatment. After many years of having lupus, patients may develop hypertension (high blood pressure), kidney failure, osteoporosis, atherosclerosis (plaque and fat build-up in the arteries) and various forms of heart and lung diseases. With proper treatment, the majority of people diagnosed with lupus have a normal life expectancy, but some will experience disability. Each patient will likely have his or her own specific pattern of symptoms and flares. The main features of lupus can change over time.
Lupus patients who are positive for antiphospholipid antibody (aPL) can develop blood clots and heart valve disease and may require additional medications, such aspirin, warfarin (Coumadin), heparin, or low-molecular-weight heparin (Lovenox or Fragmin) to reduce the risk of clots. (See also antiphospholipid antibody syndrome.)
Pregnancy is possible in most patients with lupus, but complications are frequent. Anyone with lupus should be considered to be high risk for health complications during pregnancy, especially if the disease is active. When a woman with lupus becomes pregnant or is planning to become pregnant, she should get a referral for appropriate, specialized care. Medications may need to be changed to protect the fetus. Patients with high blood pressure or kidney disease are at risk to have this worsen during pregnancy. Lupus patients who are positive for aPL are at high risk of miscarriage, while patients with anti-Ro/SSA and anti-La/SSB antibodies are at risk for delivering a child with neonatal lupus.
About one third of patients with SLE (but not those with cutaneous, drug-induced, or neonatal lupus) develop kidney disease, known as nephritis. Despite advances in therapy, a minority of patients with lupus nephritis will develop kidney failure. These patients must undergo dialysis. About one-third of patients who start dialysis during an acute lupus flare will be able to discontinue it within the first year. The remaining two-thirds, and those suffering gradual deterioration of kidney function over several years will require either continual dialysis for life or a kidney transplant.
Depending on the symptoms, blood test results and the particular organs involved, a person with lupus may receive one or more of the following:
Get more detailed information about lupus from the articles and other content below, or select Treating Physicians to find the best rheumatologist at HSS for your particular condition and insurance. The Lupus and APS Center of Excellence offers multidisciplinary patient care, support and education.