What is lupus?

Lupus is a chronic (long-lasting) autoimmune disease in which the immune system, for unknown reasons, becomes hyperactive and attacks normal tissue. This attack results in inflammation and brings about symptoms.

What does autoimmune mean?

Literally it means immune activity directed against the self. The immune system fights the body itself (Auto=self). In autoimmune diseases, the immune system makes a mistake and reacts to the body's own tissues.

What is inflammation?

Literally it means setting on fire. It is a protective process our body uses when tissues are injured. Inflammation helps to eliminate a foreign body or organism (virus, bacteria) and prevent further injury. Signs of inflammation include; swelling, redness, pain and warmth. If the signs of inflammation are long lasting, as they can be in lupus, then damage to the tissues can occur and normal function is impaired. This is why the treatment of lupus is aimed at reducing the inflammation.

What happens in autoimmune diseases like lupus?

The immune system is designed to protect and defend the body from foreign intruders (bacteria, viruses). You can think of it like a security system for your body. It contains several different types of cells, some of which function like "security guards" and are constantly on patrol looking for any foreign invaders. When they spot one, they take action, and eliminate the intruder. In lupus, for some reason and we don't know why, the immune system loses its ability to tell the difference between a foreign intruder and a person's own normal tissues and cells. So, in essence, the "Security Guards" make a mistake, and they mistakenly identify the person's own normal cells as foreign (antigens), and then take action to eliminate them. Part of their response is to bring antibodies to the site that then attach to antigens (anything that the immune system recognizes as non-self or foreign) and form immune complexes. These immune complexes help to set in motion a series of events that result in inflammation at the site. These immune complexes may travel through the circulation (blood) and lodge in distant tissues and cause inflammation there.

Where did the name come from?

Lupus is the Latin word for wolf. The term has been associated with the disease since the 10th century, though the reasons are unclear. Erythematosus means redness. It is speculated that the name was given to describe the skin lesions (sores), which typically are red and perhaps at that time in history were thought to resemble the bite of a wolf. Today we know that not everyone with lupus has rashes or skin lesions, and those who do would not say their rashes look anything like a wolf bite.

Who gets lupus?

Lupus can occur at any age, and in either sex. Nine out of ten people with lupus are women. During the childbearing years (ages 15-44) lupus strikes women 10-15 times more frequently than men.

People of all races can have lupus; however, African American women have a three times higher incidence (number of new cases) and mortality than Caucasian women. They tend to develop the disease at a younger age and to develop more serious complications. Lupus is also more common in women of Hispanic, Asian, and Native American descent.

The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) has developed a strategic plan for reducing health disparities. Lupus is included as an area of research focus. Further information on disparities in lupus and the strategic plan is available at: http://www.niams.nih.gov/an/stratplan/stratplan.htm

What are the symptoms of lupus?

Symptoms of lupus vary widely depending on the individual case and the form of lupus present. Most people with lupus do not experience all of these symptoms. The list only serves to alert people to clues that might indicate the presence of lupus in an undiagnosed person.

• Achy or swollen joints
• Persistent fever over 100 degrees
• Prolonged, extreme fatigue
• Skin rashes, including a butterfly shaped rash across the cheeks and nose
• Pain in the chest on deep breathing
• Anemia
• Excessive protein in the urine
• Sensitivity to sun or ultraviolet light
• Hair loss
• Abnormal blood clotting problems
• Fingers turning white and/or blue in the cold
• Seizures
• Mouth or nose ulcers lasting longer than two weeks

Are there different kinds of lupus?

There are four forms of lupus including:

Cutaneous lupus erythematosus affects the skin. There are many different types of cutaneous lupus including:

• Chronic cutaneous LE (CCLE) which is sometimes called discoid.
• Subacute cutaneous LE (SCLE), and
• Acute cutaneous LE (ACLE).

See: Skin Disease in Lupus

Systemic lupus erythematosus (SLE) attacks multiple systems in the body which may include: the skin, joints, lungs, blood, blood vessels, heart, kidneys, liver, brain and the nervous system.
See:  Introduction to Lupus

Drug-induced lupus may develop after taking certain prescription medications. Symptoms generally disappear, within weeks to months, after the drug is discontinued.
See: Drug-Induced Lupus Erythematosus

Neonatal lupus, a fourth type, is a rare condition. It is not the same thing as SLE.
See: Neonatal Lupus

Lupus in Overlap

The majority of people with lupus have lupus alone. Between five and thirty percent of people with lupus report having overlap symptoms characteristic of one or more connective tissue diseases. There are several well-recognized overlaps that may affect people with lupus including: lupus and rheumatoid arthritis (RA), lupus and myositis, lupus and systemic sclerosis (SSc or scleroderma), lupus and Sjogren's syndrome (SS).
See:  Lupus In 'Overlap' With Other Connective Tissue Diseases

Is lupus contagious?

No, not even through sexual contact.

Is lupus a fatal disease?

Lupus is not a universally fatal disease. In fact, today with close follow-up and treatment, 80-90% of the people with lupus can expect to live a normal life span. Lupus does vary in intensity and degree, however, and there are people who have a mild case, there are those who have a moderate case and there are some who have a severe case of lupus, which tends to be more difficult to treat and bring under control. For people who have a severe flare-up, there is a greater chance that their lupus may be life-threatening. We know that some people do die of this disease and because of that we have a tremendous amount of respect for the potential of this disease. However, the majority of people living with lupus today can expect to live a normal lifespan.

People frequently read in the literature that, 80-90% of people with lupus live for more than ten years. Unfortunately, this is often misinterpreted as- people with lupus live for only ten years. Let us clarify this.

It is important to understand that the "10 years" does not represent the number of years the person will live, but rather the number of years involved in the study. The studies followed patients with lupus from the time of diagnosis for a period of ten years. At the end of this research period they were able to conclude that 80-90% of the people enrolled were still alive. What this study did not look at is what happened in year 11, 12, 15, 20 and so on. We know there are many people who have been living with lupus for 15, 19, 25, 30 and 40 years. This is not a disease that is universally fatal to all. The majority of people with lupus today can expect to live a normal lifespan.

When people die of lupus, what do they usually die of?

Overwhelming infection and kidney failure are the two most common causes of death in people with lupus. Recently there is new information which indicates  heart disease may be another leading cause of death among people with lupus.

Lupus and cancer Is lupus a form of cancer?

No, lupus is not a form of cancer. It is an autoimmune disease.

Are people with lupus more likely to develop cancers?

People with lupus are no more likely to develop cancer than are people in the general population. However, people who have received certain chemotherapy drugs do carry the added risk of developing cancer sometime later in life.

Are there any special considerations regarding treatment of cancer in people with lupus?

Cancer can be treated in many ways; with surgery, radiation and/or chemotherapy. All people with lupus who have surgery for cancer, should be followed closely by their personal physician and/or the rheumatologist to evaluate the activity of their lupus throughout the course. For patients receiving steroid therapy and surgical procedures, their steroid dosage will have to be adjusted during the time just shortly before surgery and then tapered slowly over time after surgery. If a person is scheduled for surgery and they take non-steroidal anti-inflammatory drugs or aspirin, any of which may effect their clotting time, these medications will need to be stopped prior to surgery in order to minimize bleeding.

Chemotherapy usually doesn't present any particular problems and can in turn treat active lupus.

Is lupus like AIDS?

No. In AIDS (Acquired Immune Deficiency Syndrome) the immune system is under active; it is deficient. In lupus the immune system is overactive, producing large quantities of antibodies. AIDS is contagious but lupus is not. The majority of people with lupus can expect to live a normal lifespan.

Lupus anticoagulant - I was just diagnosed with the lupus anticoagulant. Does this mean I have systemic lupus?

The lupus anticoagulant is classified as a type of antiphospholipid antibody that was first detected in people with lupus. It was later learned that many people who do not have lupus, also produce these antibodies. In fact, according to most studies, greater than 50% of the people who have antiphospolipid antibodies, DO NOT have lupus. So, just because a person has the lupus anticoagulant antibodies does not necessarily mean that they have or will develop systemic lupus.

There is a syndrome called the "Primary" Antiphospholipid Syndrome (PAPS). This term is used to describe people who do not have any signs or symptoms of SLE but produce antiphospholipid antibodies such as the lupus anticoagulant and/or anti-cardiolipin antibodies and experience problems with blood clots, miscarriages, or thrombocytopenia (low platelets). People with PAPS do not have lupus.
See: Blood Disorders in Lupus , and Antiphospholipid Antibodies and Systemic Lupus Erythematosus

Is it possible to have antiphospholipid antibodies and not have systemic lupus?

Yes. Although these antibodies were first discovered in people who had lupus, it was later learned there are many people who produce these antibodies who do not have systemic lupus.
See:  Blood Disorders in Lupus , and Antiphospholipid Antibodies and Systemic Lupus Erythematosus

Is it possible to have antiphospholipid antibodies and not have symptoms related to them, i.e. blood clots, miscarriages or low platelets?

Yes. In fact, most people with these antibodies DO NOT and will not have symptoms related to them.

Antiphospholipid Antibody Syndrome Foundation website

Lupus and Multiple Sclerosis - I was diagnosed with MS 3 years ago and now my doctor thinks I may have lupus. Is there a connection between MS and lupus?

Multiple Sclerosis and lupus are both autoimmune diseases. They are diagnosed and treated in very different ways. There is no direct connection between the two, however, lupus, known as a great imposter, can sometimes mimic or imitate the symptoms of MS.
See: Systemic Lupus and the Nervous System

My MRI from 3 years ago showed plaques. Is there anything on the MRI of people with lupus of the nervous system that is diagnostic for CNS lupus?

Diagnosis of CNS lupus is difficult as there is not one specific diagnostic test to detect nervous system involvement in lupus. The abnormalities seen on the MRI scans are not specific for systemic lupus. In other words, they could be due to a number of things. The findings on these specialized tests and sometimes other tests such as a brain wave test (electroencephalogram, EEG) and a spinal tap have to be considered along with clinical and laboratory findings in establishing a diagnosis of CNS lupus.
See: Systemic Lupus and the Nervous System

My child has lupus. What is the prognosis?

The prognosis for children and adolescents with systemic lupus has improved dramatically over the past twenty years. With modern therapy, children do nearly as well as adults.
See:  Childhood Lupus .