Systemic Lupus Erythematosus LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE

Systemic Lupus Erythematosus LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE From the Lupus Foundation of Delaware Valley, Inc.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.

Systemic lupus erythematosus (SLE or lupus) is a disease which can affect many parts of the body, can be both acute and chronic, and can cause many different symptoms. The disease can range from very mild to very severe. Among adults, nine females will have lupus for every one male. Lupus most commonly affects people between the ages of twenty and forty and occurs, therefore, most frequently in women during their child-bearing years. Three times as many black as white women are affected. Children and teens can also get lupus.

During the first ten years of life girls will have lupus three to seven times more often than boys. (Childhood lupus is the subject of Chapter 15.)The cause of lupus is not known. The immune system, which is nature's way of protecting us from infection and cancer, works improperly in patients with lupus. Certain white blood cells, the T lymphocytes, aid in control of the immune system. In lupus, these cells fail to fulfill their regulatory function and the immune system becomes active when it is not supposed to. What starts this has not been discovered.

It appears that some foreign invader, some trigger or triggers from the outside must enter the body and set this process in motion. For years the search has gone on in vain to find a virus which could act as such a trigger. We do know that sunlight and some chemicals appear to trigger lupus in some patients, though not in all. We also know that some people have an inherited tendency to develop lupus. Something in their genetic makeup makes them especially sensitive to an environmental trigger. (Chapter 4 discusses recent advances in the genetics of lupus.)To aid in the diagnosis of lupus, the American College of Rheumatology has listed various criteria (guidelines based on symptoms and laboratory tests) to be used in identifying the disease (Table 1). The list contains the problems that are most typical of lupus as compared to other similar diseases.

It is not meant to include all, or even the most common problems a patient with lupus may experience. Persons with lupus most frequently suffer from fatigue, fevers, muscle and joint aching and stiffness, swollen glands, and generalized feelings of being unwell. None of these are listed as criteria for diagnosing SLE, however, because they occur in many other diseases as well. The first of the criteria involve the skin. The best known of the skin problems in lupus is the "butterfly" rash. This is a red rash over the cheeks and the bridge of the nose (the malar area). Several other skin-related problems which commonly occur are sun sensitivity, loss of hair, red plaques (slightly raised patches of skin that have a definite border) associated with scaling and plugging of the hair follicles ("discoid" rash), hives, dryness and ulceration of the mucous membrane (mouth, nose, and vagina), and dilated and broken blood vessels. (These are discussed in more detail in Chapter 5 and referred to in Chapters 12 and Chapter 14.) The circulation of blood to the skin and the underlying tissues is sometimes temporarily decreased in lupus, this is known as Raynaud's phenomenon.

The decrease is most commonly caused by cold temperatures but can also result from psychological stress. The tips of the fingers, the tips of the toes, and occasionally the nose and the ears are affected. These areas first turn white, then blue, and finally, as the circulation returns, red. If the circulation is reduced over a long period of time, the skin may break down and ulcerations (sores) appear.Almost all lupus patients have joint pains at some time during their illness, and many have joint inflammation (swelling, warmth, redness, pain) or arthritis. The type of arthritis associated with lupus is similar to that seen in rheumatoid arthritis but is usually not as severe and does not cause a wearing away of the bone with resulting deformity. It most commonly affects the small joints of the hands, the wrists, the knees, and the feet. Sometimes patients with lupus, especially those on corticosteroids, have involvement of the bone due to a loss of blood supply.

When this occurs in the bone near a joint, degenerative arthritis may result. (See Chapter 13.)Serositis is the inflammation of the delicate tissues which cover certain internal organs and line body compartments. It is quite common in lupus, most often occurring as inflammation of the covering of the lung and the lining of its compartment in the chest (pleuritis), and as inflammation of the covering of the heart (pericarditis). Movement of the involved tissues may cause chest pain, and the doctor can often hear a characteristic "rub" when listening over the inflamed areas with a stethoscope. Abdominal pain in lupus is sometimes caused by inflammation of the covering of the intestines and the lining of the abdominal cavity (peritonitis), and can imitate pancreatitis, diverticulitis, appendicitis, etc. Fluid sometimes accumulates as a result of serositis and may have to be withdrawn by a needle. In the case of pericarditis, an operation to make an opening in the covering of the heart (a "Pericardial window") may be necessary to relieve pressure on the heart from this fluid.One of the most feared complications of lupus is involvement of the kidney.

It is present to some degree in about 50% of cases. It can vary from very mild involvement which causes the patient no symptoms, to severe disease leading to kidney failure and the need for dialysis or kidney transplantation. Doctors detect early kidney problems by discovering protein or blood cells in the urine. They frequently ask the patient to collect 24 hour urine samples so as to be able to make better estimates of the degree to which the kidney is functioning. At times it is necessary to obtain a biopsy of the kidney to look for the presence of antibodies and kidney cell damage. (Lupus and the kidney is further discussed in Chapter 6.)The central nervous system (CNS) may become involved by systemic lupus erythematosus. Such involvement may cause a variety of disorders including seizures, psychosis, paralysis and personality disorders.

Since very similar problems might be the result of infection, hardening of the arteries, or mental illness, etc., and since there is no one test for central nervous system lupus, these disorders may be difficult for the physician to diagnose. (Neurologic problems seen in lupus are discussed in detail in Chapter 7, neuropsychological testing is discussed is Chapter 8 and depression in lupus is discussed in Chapter 10.)One of the frequent disorders in lupus is a decrease in the number of white blood cells, especially the lymphocytes. Since some of the drugs used to treat lupus can also cause a decrease in the white blood cell count, monitoring the safe use of these drugs can, at times, be difficult.

The platelet is another blood cell frequently decreased in lupus. Platelets play a key role in blood clotting and having too few platelets can lead to serious bleeding disorders. Anemia, a decrease in the number of red blood cells, is almost always present in cases of active lupus. It may result from bleeding, from a bone marrow which is depressed by illness and therefore does not produce enough red blood cells, or from the destruction of red blood cells by antibodies. (More about the anemia of lupus in Chapter 11.) When the number of certain T lymphocytes is decreased, the B lymphocytes of the lupus patient are stimulated to manufacture many different antibodies. Among these are antibodies called "autoantibodies" which target the lupus patient's own tissues. The body, in essence, attacks itself. In addition to the widely known antinuclear antibody (ANA), there are many other antibodies, the presence of which aid in the diagnosis of lupus. (See Chapter 2 for a discussion of ANA, anti-DNA, anti-Sm, anti-Ro, anticardiolipin, etc.)Certain drugs in common use may cause antinuclear antibodies to develop and, therefore, cause a mild form of lupus known as drug-induced lupus.

Drug-induced lupus is characterized by muscle pain and arthritis, fever, and pleuritis. Kidney and central nervous system involvement typically do not occur. Drugs which have been associated with drug-induced lupus include antibiotics, certain thyroid medication, and drugs used to control blood pressure, seizures, and heart rhythm. Two of the most common offenders are hydralazine (Apresoline) and procainamide (Pronestyl). Because of these drug effects, it is important to know what medications a patient diagnosed as having lupus has been taking.Pregnancy is the occasion for special concern in the patient with systemic lupus erythematosus.

While pregnancy does not appear to affect the overall life expectancy of the lupus patient, lupus may flare during pregnancy and there is an increase in premature delivery and in stillbirth among lupus patients. Therefore, it is very important for the woman with lupus to seek medical help early in her pregnancy from an obstetrician who is experienced in dealing with high-risk pregnancies. (This topic is covered in Chapter 18.) Lupus may be difficult at times to diagnose and to distinguish from other connective tissues diseases and the symptoms may actually overlap with some of them. Rheumatoid arthritis involves a similar, though usually more severe, arthritis with morning stiffness.

Patients with rheumatoid arthritis may have a positive ANA in addition to a positive test for an antibody known as the rheumatoid factor. Scleroderma (systemic sclerosis) typically is associated with Raynaud's phenomenon, arthritis and a positive ANA but is characterized by the development of remarkably tight skin. Dermatomyositis involves a facial rash and may be associated with arthritis and lung disease, but profound muscle weakness also occurs. Overlapping symptoms of lupus, derrnatomyositis, and scleroderma is not uncommon. Some of these overlap cases (including those referred to as mixed connective tissue disease) respond well to treatment and do not develop significant kidney or neurologic disease.Some patients with lupus require very little treatment.

They may need only steroid skin creams or ointments and sunscreens. Other patients may require the use of simple pain medications and mild anti-inflammatory medicines such as aspirin and NSAIDS (nonsteroidal anti-inflammatory drugs) for muscle pain or arthritis. Drugs such as hydroxychloroquine (an anti-malarial medication) are used for patients with arthritis and skin disease. Adrenal corticosteroids (steroids) given by mouth are usually reserved for the more serious cases such as those with kidney or central nervous system disorders, certain blood cell problems, or serositis. To supplement steroids or to replace them when they don't work, immunosuppressive drugs, which were originally developed for use in cancer chemotherapy but have also been found to be helpful in treating lupus, are used. (These and other treatments are dealt with further in Chapter 3.)

In addition to medical treatment, there are other important considerations in the treatment of lupus. Clubs and support groups are available to offer education and psychological help. (See Chapter 19.) Physical and occupational therapy and other methods used by rehabilitation medicine can provide pain relief and can help to maintain functioning (discussed in Chapter 16). Psychotherapy and counselling can be an important addition to other forms of treatment and are available from different sources including psychiatrists, psychologists, and social workers. (These are mentioned in connection with depression in Chapter 10 and with sexual dysfunction in Chapter 17.)

Finally, one of the most helpful aspects of the treatment program is a good doctor-patient relationship. This will be the subject of an entire chapter later on (Chapter 20).Over the years, we have had more and more success in the treatment of lupus. Studies done at Johns Hopkins University in 1954 showed a survival rate less than 50% after four years. Several large studies done in the 1980's, however, revealed an approximate 87% five year survival and an approximate 78% ten year survival. A smaller study from Holland published in 1989 found an even better ten year survival of 87%! There is good reason to believe that this upward trend will continue in the future as we learn more about the disease and how to control it.
=======================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.