What is Lupus?
Lupus is a chronic disease with a variety of symptoms caused by inflammation in one or more parts of the body. It is estimated that it affects over 1:1000 Canadians.
Lupus is not contagious and is not related to AIDS or cancer. It belongs in the family of diseases that includes rheumatoid arthritis, multiple sclerosis, juvenile diabetes, and scleroderma.
The most common type of lupus is SLE (systemic lupus erythematosus). It is a complex and baffling condition that can target any tissue or organ of the body, including skin, muscles, joints, blood and blood vessels, lungs, heart, kidneys, and the brain.
There are other types of lupus which mainly affect the skin. A few individuals develop drug-induced lupus as a response to some medications used to treat other conditions. These symptoms disappear when the person stops taking the medication.
Who gets lupus? Anyone can: women, men, children. Between the ages 15 and 45, eight times more women than men get lupus. In those under 15 and over 45, both sexes are affected equally.
Other Types Of Lupus Include:
Discoid lupus erythematosus (DLE) and subacute cutaneous lupus (SCLE) are diseases where skin rashes and sun sensitivity are the main problems; involvement of the internal parts of the body does not occur and life is not threatened. However, both DLE and SCLE may, at times, occur along with the systemic form of lupus. Performing appropriate tests to rule out systemic lupus erythematosus is therefore important when DLE or SCLE is newly diagnosed.
Neonatal lupus is an uncommon form of lupus that affects the newborn child. This most often occurs in the children of women with SCLE or systemic lupus who also have a particular antibody (molecule that recognizes other molecules that are foreign to the body) in their bloodstream. Skin rashes that come and go and a decrease in some of the blood cells may occur but these problems go away within six months after birth. In rare cases, the tissues that control the normal heartbeat may be affected. However, this serious problem can often be corrected with a pacemaker.
Drug-induced lupus is triggered by certain drugs. Only a small number of people taking these drugs develop this form of lupus which has many of the symptoms of systemic lupus. The most important feature of drug-induced lupus is that it goes away when the offending drug is stopped.
Systemic lupus erythematosus SLE (or simply “lupus”) is a treatable, chronic, autoimmune, inflammatory disease that can affect any organ in the body and in a pattern that varies greatly from person to person. Lupus is characterized by autoantibodies. SLE is a chronic illness, which means that the disease is lifelong (however, most persons with SLE will not be continuously sick for the rest of their lives). Autoimmune means that there is a disorder of the immune system which cannot tell the difference between the person’s own tissues and foreign tissues. This conflict leads to inflammation (the normal body response to injury or infection) in various organs which causes the symptoms of lupus to appear. If severe or untreated, this inflammation may cause organ damage and loss of function. Autoantibodies, meaning antibodies directed against one’s self, are involved in this process and are typical of SLE.
Is there a cure?
What is known is that lupus is an autoimmune disease. The immune system is the part of the body that protects us against germs such as bacteria and viruses. In the normal individual, certain cells of the immune system make proteins called antibodies that react with foreign substances in the body and destroy them. In lupus, something goes wrong with the immune system so that it also makes antibodies that attack the person’s own tissues. The result is an autoimmune reaction which causes the inflammation that affects the specific tissues or organs in SLE.
Who can get lupus?
Women of child-bearing age (15 to 45) are most often affected. Indeed, in that particular age group, lupus is 8 to 13 times more common in women than in men. However, the disease does occur in men, in children and in the elderly. According to the Lupus Foundation of America, of the 500,000 Americans estimated to have SLE, the disease occurs in 1 of every 600 white women of child-bearing age and in 1 of every 200 black women. Lupus is therefore not rare and is more common than multiple sclerosis, muscular dystrophy or leukemia. Lupus is diagnosed worldwide. In Canada, estimates of the number of lupus patients range from 15,000 (based on the number of patients followed in various University Hospital Lupus Clinics throughout the country) to 50,000 (based on the figures used by the Lupus Foundation of America, adjusted to the Canadian population). At any rate, it is clear that SLE is not a rare disease in Canada.
Lupus is not contagious.
Lupus is not an infection. It is not contagious, meaning that it cannot be passed from person to person like a cold, the measles or the flu. It is not a form of cancer. There is absolutely no relationship between lupus and AIDS.
Is lupus hereditary? Is there a cure?
Heredity may influence whether or not a person develops lupus. It is not unusual for a lupus patient to have in his or her family a relative with an autoimmune disease (including lupus). In fact, SLE studies have shown that between 0.4 to 5 per cent of the relatives of lupus patients may themselves develop lupus. While this may sound frightening, remember that the odds of not having lupus when someone else in the family has it are at least 95 per cent or greater.
As yet, there is no cure for lupus. However, researchers studying this disease have made tremendous advances in our knowledge of SLE and this research is continuing. Patients are fully justified in their hopes that a cure will be found.
How does lupus affect life expectancy?
An extraordinary improvement in life expectancy has occurred in SLE. Just 35 years ago, at the famous Johns Hopkins University, only half of all lupus patients were alive four years after diagnosis. However, by the late 1970’s, 80 to 90 per cent of all lupus patients were alive after 10 years. (To date, no studies of survival after ten years have been completed.)
This shows the dramatic improvement in the survival rate for patients diagnosed in the sixties and seventies. Today, we believe that the survival of lupus patients has improved even further. Several factors have combined to cause this remarkable improvement. Earlier diagnosis, recognition of milder forms of the disease and better use of tests have all helped. However, in my view, the single most important factor has been the discovery and use of prednisone in the treatment of SLE.
In summary, systemic lupus erythematosus is a chronic autoimmune disease that affects primarily women of child-bearing age. It can be mild or serious and often goes into remission and remains quiet for long periods of time. Treatment is successful at bringing the symptoms under control in almost every case. The next chapter will discuss the many symptoms of lupus and will help you to understand how your physician has determined that “you have lupus”.
Hopeful facts about systemic lupus erythematosus (SLE)
- If you have been told recently that you have SLE, it is important at this point to be aware of the following facts which bring great hope to lupus patients:
- Lupus can be a serious disease but it is quite clear that milder forms of the disease do exist and are being increasingly recognized.
- Lupus is treatable and much can be done to control it. In fact, with proper treatment it is the rule, not the exception, for physicians (and patients) to succeed in bringing lupus under control.
- Many patients undergo cycles in which the disease becomes quiet after it is brought under control. This symptom-free period is called remission and may last several years.
- Better methods of diagnosis, treatment and follow-up have improved the life expectancy of lupus patients so that nowadays lupus is seldom seen to be fatal.
A brief history of lupus
Lupus has been known as a disease for over 2000 years. Hippocrates, who lived from 460 to 370 BC, first described lupus lesions (lesions means damage). The name “lupus” comes from a Latin word meaning wolf and was used in reference to the re ulcers on the face. In 1851, Cazenave introduced the name “lupus erythematosus”. Later in the 1800’s, Kaposi described the butterfly rash on the face and Sir William Osler, a Canadian physician, expanded the concept of lupus as a “systemic” disease, a disease which may affect many parts of the body.
In 1948, Hargraves and his colleagues discovered a particular blood cell that appeared in lupus patients and lupus began to be considered as an autoimmune disease. In 1957, Friou described the antinuclear antibodies (antibodies which react with the nucleus of cells) which confirmed that lupus is indeed a disorder of the body’s immune system.
With a clearer understanding of the disease process at work in SLE, researchers have continued their efforts to discover a cause and cure for lupus. In the last 30 years, enormous advances have been made that have improved both the diagnosis and treatment.