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Lupus Summary


What is lupus?

Lupus Stats

  • Frequency: 30 - 50 new cases every year per 100,000 people.
  • In the USA, roughtly 2 Million people have Lupus. More people have lupus than cerebral palsy, cystic fibrosis, and MS combined
  • Mortality: 10 year survival rate 76-90%; 90% live a normal life span
  • Sex Bias: women are 9x more likely to develop lupus then men.
  • Race Bias: 1 out of 600 Caucasian women develop Lupus; 1 out of 200 African-American women develeop Lupus

Lupus erythematosus is a chronic, inflammatory, multisystem disorder of the immune system. “Lupus” means wolf and Erythematosus means “redness.” Doctors coined this name for the disease because they thought the facial rash that frequently accompanies lupus looked like the bite of a wolf.

In lupus, the body develops antibodies that react against the person’s own normal tissue. Lupus is thus an autoimmune disease.These antibodies are markers for lupus, and are one indicator of many immune system abnormalities that lead to clinical manifestations. The course is unpredictable and individualized; no two patients are alike. Lupus is not contagious, infectious, or malignant.

There are four categories of lupus: systemic lupus erythematosus (SLE), cutaneous lupus, drug-induced systemic lupus erythematosus, and neonatal lupus.

SLE is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well

What are the symptoms of lupus?

Lupus can affect many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Although people with the disease may have many different symptoms, some of the most common ones include extreme fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes, and kidney problems.

Lupus varies greatly in severity, from mild cases requiring minimal intervention to those in which significant and potentially fatal damage occurs to vital organs such as the lungs, heart, kidneys, and brain. For some patients, the disease can be characterized by “flares” of activity interspersed with periods of improvement or remission.

A flare, or exacerbation, is increased activity of the disease process with an increase in physical manifestations and/or abnormal laboratory test values. Periods of improvement may last weeks, months, or even years. Other patients have continuous, or chronic, activity. Although remissions are unusual, some patients never develop severe manifestations, and the outlook is improving for patients who do develop them.

Typical Lupus Butterfly Rash (Malar)
butterfly rash

 

Who gets lupus? It usually develops in young women of childbearing years, but many men and children also develop lupus. SLE also appears in the first-degree relatives of people with lupus more often than it does in the general population, which indicates a hereditary component. However, most cases of lupus occur sporadically, indicating that both genetic and environmental factors play a role in the development of the disease.

What are the risk factors for lupus? Lupus is 9x more common in women than men. Lupus is three times more common in African American women than in Caucasian women and is also more common in women of Hispanic, Asian, and Native American descent. In addition, lupus can run in families, but the risk that a child or a brother or sister of a patient will also have lupus is still quite low. Lupus is most frequently diagnosed between the ages of 15 and 45.

What causes lupus? The cause of lupus is unknown, but it has been associated with genetic, environmental, and infectious causes.

What is the prognosis for lupus? 90% of lupus patients diagnosed live a normal life span with the appropriate treatments and care of their physicians along with their own compliance as self advocates in their medical care, medications and treatments involved with their systemic illnesses.

What is Cutaneous Lupus? Cutaneous Lupus affects primarily the skin, is common among patients with lupus erythematosus. The most prevalent and severe form of cutaneous lupus is called chronic cutaneous lupus. It is commonly known as discoid lupus, but it has other forms as well.

Discoid lupus erythematosus is a chronic skin disorder in which a red, raised rash appears on the face, scalp, or elsewhere. The raised areas may become thick and scaly and may cause scarring. The rash may last for days or years and may recur. A small percentage of people with discoid lupus have or develop SLE later.

What is Drug-induced Lupus Erythematosus? Drug induced lupus develops after the use of certain drugs or biologics and has symptoms similar to those of SLE. The characteristics of this syndrome are pleuropericardial inflammation, pleuritic chest pain (inflammation of the lining of the lungs), pericarditis (swelling and irritation of the pericardium, the thin sac-like membrane that surrounds the heart), fever, rash, and arthritis. Serologic (Blood) changes can occur. The clinical and serologic signs usually subside gradually after the offending drug is discontinued. A wide variety of drugs is implicated in this form of lupus, including:

Drugs Implicated as Activators of Drug-Induced Lupus

  • Isoniazid
  • Hydralazine
  • Procainamide
  • Minocycline
  • Anti-TNF biologics

More recently, the newer TNF (tumor necrosis factor) inhibitors used to treat rheumatoid arthritis, Crohn’s disease, and ankylosing spondylitis have been associated with the development of lupus symptoms. As with lupus triggered by other drugs, the symptoms resolve when the agent is stopped.

What is Neonatal lupus? Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE, Sjögren's syndrome, or no disease at all. Scientists suspect that neonatal lupus is caused by autoantibodies in the mother's blood called anti-Ro (SSA) and anti-La (SSB). Autoantibodies ("auto" means self) are blood proteins that act against the body's own parts. At birth, the babies have a skin rash, liver problems, and low blood counts. These symptoms gradually go away over several months. In rare instances, babies with neonatal lupus may have a serious heart problem that slows down the natural rhythm of the heart. Neonatal lupus is rare, and most infants of mothers with SLE are entirely healthy. All women who are pregnant and known to have anti-Ro (SSA) or anti-La (SSB) antibodies should be monitored by echocardiograms (a test that monitors the heart and surrounding blood vessels) during the 16th and 30th weeks of pregnancy. It is important for women with SLE or other related autoimmune disorders to be under a doctor's care during pregnancy. Physicians can now identify mothers at highest risk for complications, allowing for prompt treatment of the infant at or before birth. SLE can also flare during pregnancy, and prompt treatment can keep the mother healthier longer.

lupus doctor

 

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