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Lupus (SLE)

What is lupus?

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 frequently 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 and offers a dedicated team of specialists and resources at the Lupus and APS Center of Excellence.

Lupus facts

Lupus Infographic

 

What are the different types of lupus?

There are five recognized forms of lupus:

  • Systemic lupus erythematosus (SLE) is the disease most commonly mentioned, and the most serious since it involves the entire body.
  • Discoid lupus is a skin-only illness in which a specific rash, mostly a scarring rash of circular-shaped lesions, occurs without other symptoms (SLE patients sometimes have the same rash).
  • Subacute cutaneous lupus describes a more widespread rash that is often worsened by sun exposure. It is usually associated with a limited form of SLE.
  • Drug-induced lupus is relatively rare and is triggered by certain drugs, such as hydralazine and some anti-seizure drugs. Drug-induced lupus usually causes rash, arthritis, chest pains associated with heart and lung inflammation, and blood test abnormalities. It goes away if the drug is stopped.
  • Neonatal lupus occurs in infants whose mothers have specific blood abnormalities, but not in infants of mothers with SLE that do not have these abnormalities. It consists mainly of a transient, unimportant rash and blood test abnormalities that disappear within a few months, and rarely leads to a serious form of heart disease.

What are the symptoms 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:

  • Arthritis
  • Malaise, fatigue, fevers, weight loss
  • Rashes (often in response to sunlight exposure), usually located on the face, neck, chest, backs of the upper arms, fingertips and/or base of the fingernails
  • Hair loss
  • Swollen lymph nodes
  • Kidney (renal) disease
  • Chest pains due to pleuritis or pericarditis
  • Abdominal pain
  • Headache, mood disorder, confusion, psychosis, seizure or stroke
  • Photosensitivity – in which sun exposure makes you ill (beyond just a rash), requiring the use of a high-grade sunscreen
  • Small, painless ulcers in the mouth and nose
 

Patients with lupus also characteristically have the following abnormal lab results:

  • Antibodies against your own cells. Almost all have antinuclear antibodies (ANA).
  • One or more of the following which are subtypes of ANA and related antibodies: anti-dsDNA, anti-Sm, anti-Ro/SSA, anti-La/SSB, anti-RNP, antiphospholipid antibodies (anticardiolipin, lupus anticoagulant)
  • Blood (hematologic) abnormalities, such as leukopenia (low white blood cell count), lymphopenia (low lymphocyte count), thrombocytopenia (low platelets), and various forms of anemia, including autoimmune hemolytic anemia
  • Evidence of inflammation in the blood, usually measured by an increased erythrocyte sedimentation rate (ESR)

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.

What are the health complications of lupus?

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.

  • Are people with lupus at a higher risk for blood clots?

    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.)

  • Can I get pregnant if I have lupus?

    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.

  • Does lupus lead to kidney disease?

    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.

What are the treatments for lupus?

Depending on the symptoms, blood test results and the particular organs involved, a person with lupus may receive one or more of the following:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Hydroxychloroquine (Plaquenil)
  • Corticosteroids
  • Immunosuppressive drugs such as azathioprine (Imuran), methotrexate (Rheumatrex), cyclosporine (Sandimmune, Neoral), and mycophenolate mofetil (CellCept), cyclophosphamide (Cytoxan) and tacrolimus (Prograf). Some are given by injection or intravenously.
  • Biological agents such as belimumab (Benlysta), rituximab (Rituxan), TNF alpha inhibitors (Enbrel, Humira,Cimzia, and others)
  • Other biological agents now considered experimental, including those that block, interferon and other cytokines
  • For special circumstances, intravenous immunoglobulin, and vitamins/antioxidants, and drugs used for other purposes but likely to be effective (such as eculizumab [to inhibit complement], angiotensin converting enzyme inhibitors [to control blood pressure and protect kidneys]), antidepressants, anti-seizure medications.

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.

Lupus overview articles

Lupus Top 10 Series

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