LUPUS/ SLE Essay, Research Paper

SYSTEMIC LUPUS ERYTHEMATOSUS

“HOPE IS GROWING”

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease which may affect many different organs and tissues in the body. Women of child bearing age are typically affected, but individuals of any age, sex, or race may develop the disease. SLE while uncommon, is not rare, with an estimated disease prevalence of 1 in every 2,000 population. It is a condition which appears to be increasing in prominence especially over the last 15 to 20 years. This is likely explained by the earlier recognition of milder cases because of increased patient and physician awareness and by the enhanced availability of sensitive laboratory tests helpful in the diagnosis. Although the exact cause is not known, most of the features of the disease seem to be due to a fundamental abnormality of the body’s immune system.

The immune system is the body’s defence mechanism against foreign substances entering the body. It depends on the formation of compounds called antibodies and on hite cells called lymphocytes which rise to the defense of the body in case of invasion by foreign agents such as germs or viruses. This is a normal and desirable process in the healthy individual.

In patients with SLE, there seems to be a defect in the body’s immune system whereby antibodies are mistakenly formed against the body’s own tissues. This leads to inflammation and damage in the tissues so affected. Patients with SLE can be identified by the presence of these abnormal antibodies in their blood stream.

It is not clear what triggers this immune abnormality but several factors seem to be contributory in some patients. These include infection, hormonal, genetic, and unidentified environmental factors. Some drugs including those used for the treatment of tuberculosis (isoniazid), high blood pressure (hydralazine), and convulsions (dilantin) have also occasionally been associated with the development of SLE.

Clinical Features

The majority of patients with SLE have very mild symptoms which can be easily controlled with simple measures. A small minority have more serious manifestations which may require more aggressive forms of treatment.The seriousness of the disease is frequently related to the type and number of organs affected. The following is a summary of some of the signs and symptoms that may occur in lupus patients grouped according to the organs or tissues affected:

General symptoms

Fever and unusual fatigue occur in up to 80 or 90% of SLE patients at some time during the course of their illness.

Skin rash a very common feature occurring in many patients. The classic rash is called a “butterfly rash” because it occurs in a butterfly-like patch over the bridge of the nose and cheeks. This type of rash is in fact quite uncommon with most lupus rashes being far less specific and occurring anywhere on the body but especially over sun exposed areas. Many lupus rashes appear to be provoked or aggravated by direct sun exposure.

Sores may also occur in the nose and mouth, and scalp hair loss may occur in some individuals.

In a closely related condition called discoid lupus erythematosus (DLE), the rash may arise as distinct scaly and reddish patches which may heal with scarring. Patients with DLE are frequently otherwise well. They demonstrate few, if any, of the symptoms of SLE and usually have a nearly normal laboratory profile.

Joints – stiffness, pain, and swelling may commonly occur. Unlike rheumatoid arthritis however, permanent damage to the joints is almost unheard of.

Membranes of the heart and lungs, the linings of the heart and lungs may occasionally become inflamed in SLE patients leading to sharp chest pains and shortness of breath. If it involves the lung, the condition is called pleuritis. If it affects the heart, the condition is called pericarditis.

Blood cells – a number of abnormalities may occur in the blood including anaemia or a fall in the red blood cell count and/or falls in the white cell count or platelet count (particles in the blood that help with clotting) and thus lead to potential problems with bleeding.

Kidneys – often a sign of more serious disease, inflammation of the kidney may lead to loss of protein in the urine, increased blood pressure and occasionally kidney failure.

Brain and nerves – fortunately, a relatively rare problem, patients so affected may have trouble with headaches convulsions, emotional disturbances, weakness or numbness of the extremities.

Diagnosis

The diagnosis of SLE is suspected in any individual who presents with one or more of the clinical features outlined above. A diagnosis is confirmed by laboratory tests which show the presence of one or more abnormal circulating antibodies in the blood stream. These antibodies may be directed against any tissue in the body. The most important of these however, is an antibody directed against the centre or nucleus of the cells in the body, the so-called anti-nuclear antibody or ANA. ANAs are normally not present or present only in barely detectable quantities in healthy individuals. Thus, this test is very helpful to the doctor if he is suspicious about the possibility of SLE.

It is very important however, to stress that the presence of ANAs doesn’t specifically point to a diagnosis of SLE since abnormal antibodies of this type may occur in other conditions such as rheumatoid arthritis, certain infections and inflammation of the liver.

Thus the diagnosis of SLE requires both the presence of abnormal antibodies (especially ANAs) as well as signs and symptoms suggesting inflammation of several organs or tissues in the body. Although, all lupus patients have elevated levels of ANA, not all people with elevated ANA have lupus. Increased levels of ANA generally indicate that the physician should follow up with an anti-DNA antibody test.

To assist in the diagnosis of SLE, the American Rheumatism Association (ARA) in 1982 adopted a set of criteria for the classification of this disease. (See Table 1) It should be noted that while a variable number of these features may occur during the course of the disease, they need not occur at the same time. Moreover, it is quite unpredictable as to which patient may develop which particular symptom or grouping of symptoms at any particular time.

Management and Treatment

The management of patients with SLE has three important components:

Education of the patient and family,

Medical treatment

Follow-up.

Education of the Patient and Family

Educating the patient and family is essential in the management of SLE. Patients and family are frequently misinformed about the disease and the time taken to correct and clarify any early misconceptions is greatly rewarding. A great deal of helpful information and support by persons similarly affected is also available through patient self-help groups such as the Lupus Society. It is also important for patients with SLE to realize the importance of good health habits in the control of their illness. This includes a well balanced diet, adequate rest, as well as physical activities tailored to the individual’s tolerance. Most patients should also reduce direct sun exposure and consider the use of effective sun screen preparations. Preparations with a sun protection factor (SPF) of at least 15 and which block both UVA and UVB rays are recommended.

Medical Treatment

SLE is typically a disease which shows a fluctuating course characterized by long periods of relative inactivity (remission) punctuated by unpredictable flares of inflammation involving one or more organ systems (exacerbation). Fortunately for a majority of SLE patients, the symptoms are so mild as to require little or no specific treatment. The choice of therapy will therefore depend both on the nature as well as the severity of the symptoms. The initial treatment of minor joint aches and pains may often consist of simple anti-arthritic medications sometimes known as non-steroidal anti-inflammatory drugs or NSAIDs.

Skin rash can frequently be managed by avoidance of sunlight as well as the use of topical steroid creams as directed by the family physician. If the rash or arthritis is more troublesome, your doctor may consider a class of drugs still used for the treatment of malaria such as hydroxychloroquine or Plaquenil.

For patients with more serious symptoms such as severe fever, pleurisy or pericarditis, or falling blood count, it may be necessary to resort to the use of corticosteroids by mouth for a variable length of time. Unfortunately corticosteroids have a variety of side effects and your doctor will endeavour to taper and reduce the dosage as quickly as is medically possible.

A relatively new approach called pulse steroid therapy involves the administration of very large doses of corticosteroid either orally or intravenously over a short period such as 1-3 days. Pulse steroid therapy would seem to have the advantage of being relatively free of immediate and long term side effects.

For the rare patient where steroids are inadequate, treatment is available with a number of more potent drugs specifically directed at suppressing the formation of the abnormal antibodies which occur in SLE. These drugs, examples of which include Imuran and Cyclophosphamide, are called cytotoxic or immunosuppressive agents. These drugs are frequently effective but may have serious side effects including the suppression of the body’s normal ability to fight infection.

An alternate non-drug approach to the management of SLE, especially severe kidney disease, may be sometimes considered. This procedure, which is called plasmapheresis, involves an exchange type transfusion whereby the red blood cells are removed from the blood and returned to the body while undesirable antibodies and complexes and the liquid part of the body’s blood (plasma) are discarded. This treatment seems most effective when combined with one of the cytotoxic or immunosuppressive drugs.

Follow-up

As the course of SLE may be unpredictable, close medical follow-up is essential. This involves periodic assessment of disease activity by clinical history, physical examination and specific laboratory tests ordered by your doctor. Close follow-up during pregnancy and the immediate postpartum period is especially important as the risk of disease flare is increased during these periods. Inactive disease at the time of conception is associated with the best prognosis for both mother and baby.

There is a slight but definite increased risk of miscarriage in mothers with lupus and their babies may have an increased chance of being born premature or with low birth weights. Babies born to lupus mothers may also be at increased risk of developing lupus (neonatal lupus). This is probably due to the passage of abnormal antibodies through the placenta into the fetal blood stream. Neonatal lupus almost always resolves within 4-6 months of delivery.

Babies born to lupus mothers may also be at increased risk of being born with an abnormality to the conduction system of the heart muscle. This may or may not be associated with other signs of neonatal lupus and seems to correlate closely with a very specific type of antibody in the mother’s circulation called the anti-Ro antibody.

Conclusions

Although there is no specific cure for SLE, there is much room for optimism. Patients are now being identified far earlier and therapy, if necessary, is generally more effective. Moreover, as physicians have gained experience with the diseaseand its management, many of the past complications which often as not arose from overzealous treatment can now be avoided. More importantly, fundamental advances are being made in the understanding of the specific immune abnormalities in SLE, which undoubtedly will lead to steady improvements both in the management and the overall prognosis.

TABLE 1 ? 1982 Revised ARA Criteria for Classification of SLE*

1.Butterfly rash

2. Discoid rash

3.Sun sensitive rash

4.Mouth ulcers

5.Arthritis

6.Pleuritis/Pericarditis

7.Kidney disorder

8.Brain or nerve disorder

9.Blood disorder

10.Presence of one or more antibodies to various body tissues

11.Presence of anti-nuclear antibodies (ANA)

For the purpose of identifying patients, a person shall be said to have SLE if any 4 or more of the 11 criteria are present serially or simultaneously.

Lupus Erythematosus Society of Saskatchewan – L.E.S.S.

The Lupus Erythematosus Society of Saskatchewan Inc. is a voluntary association which is concerned about those people who are affected by Systemic Lupus Erythematosus. The primary goal of L.E.S.S. Inc. is to help its members become more informed about lupus so that through information they might assist in controlling their illness and learn to cope with the emotional effects of being chronically ill. To further this aim, we sponsor meetings in several locations throughout the province, with a variety of professional speakers presenting information about lupus, current research or specific lupus problems and their treatments.

In addition, we publish four newsletters each year which include reprints of reputable and current articles, summaries of guest speaker presentations, and information about our group and its members. We also attempt to provide opportunities for members to meet and share in a personal way as well as to offer support to those who are in need.

Lupus is not rare and in Saskatchewan there are approximately 500 people who have the disease, either diagnosed or undiagnosed. To assist in supporting these people, L.E.S.S. Inc. has groups currently operating in Saskatoon, Regina, Swift Current, Prince Albert and Hudson Bay, with contacts in Moose Jaw and North Battleford. If you are interested in obtaining information about lupus or about L.E.S.S. Inc., we would be pleased to hear from you.

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The Oxford Textbook of Rheumatology.

This 1500 textbook on immune disorders covers all phases of lupus and other associated immune disorders such as a sclerodoma, rheumatoid arthritis, Sjorjens syndrome, Chronic Fatigue Syndrome, Fibromyalagia, etc.

The New England Journal of Medicine (published weekly)

has had many excellent articles concerning lupus and other immune disorders and it references other sources of information in its bibliography.

Chronic Fatigue List

There is also a very nice Chronic Fatigue List which publishes a newsletter which information about fibromyalagia and cfs. Subscribe by E-mail to [email protected].