LUPUS AND YOU

DEFINITION and DESCRIPTIONS

Lupus or SLE (Systememic Lupus Erythematosus) is a chronic, life-long,potentially fatal autoimmune disease. This disease is characterized by unpredictable exacerbations (flare-ups) and remissions. SLE is an autoimmune disease characterized by immune dysregulation resulting in the production of antinuclear antibodies (ANA), immune complexes and the activation of the complement system. SLE is a complex disorder affecting a younger population with many similarities of HIV infection in regards for multiple organ involvement, the potential of life-threatening episodes and the need for hi-tech monitoring. By definition, Lupus or SLE is a disease in which the body's immune system turns on itself. By turning on itself, the body prepares to fight an invisible invader and thus attacks itself. This systemic autoimmune disorder produces a chronic inflammatory disease affecting all organ systems. The etiology of SLE remains unknown. A genetic predisposition, sex hormones and triggers from the environment may likely result in the dis-ordered immune response that is seen in the disease.

CHARACTERISTICS, SIGNS AND SYMPTOMS>

There are many clinical features that might be presented leading to a diagnosis of Lupus or SLE. Some of these include the following:

  • 80% of patients present with some involvement of the skin or joints.

  • Photosensitivity is common (being sensitive to sunlight)

  • Alopecia (hair loss) is also commonly seen

  • Arthritis or joint pains which can be easily confused with rheumatoid arthritis

  • FUO (Fevers of unknown origin)

  • Kidney problems such as glomerulonephritis (which can affect 50% of all patients) Also includes: heamaturia (blood in the urine), proteinuria (protein in the urine), hypertension (high blood pressure)

  • CNS disturbances such as neuropsychiatric disorders, seizures, strokes, epilepsy, and frank psychosis, depression is common

  • Blood disorders such as thrombocytopenia (low red blood cells) or some types of anemia

  • Butterfly rash on the face known as a malar rash

    HOW LUPUS IS DIAGNOSED

    As you can see, Lupus can and does strike many different organs and involves many organ systems of the body.

    Due to this involvement, Lupus is very difficult to diagnose and maybe mis-diagnosed for a length of time. Diagnosis is based on the following criteria of which 4 (four) of the 11 listed MUST be present. The criteria includes:

  • 01. Malar rash (butterfly rash of the face)

  • 02. Discoid rash (scaly rash of the scalp/face/neck/chest or arms)

  • 03. Photosensitivity

  • 04. Oral ulcers of the mouth

  • 05. Arthritis

  • 06. Serositis

  • 07. Renal disease (Kidney diseases)

  • 08. Neutropenia(<4,000 WBC"white blood cells)

  • 09. Neurologic disease

  • 10. Elevated ANA (anti-nuclear antibodies)

  • 11. Positive LE prep, anti-DNA, anti-smith antibody or false-positive STS

    Other diagnostic tools:

    LABORATORY FINDINGS:

  • 01. ANA: anti-nuclear antibodies: are present in almost all lupus patients but not specific to SLE. Antibodies to double-stranded DNA almost unique to SLE. Anti-smooth muscle antibodies are also almost exclusive to SLE

  • 02. Blood abnormalities: anemia which accounts for almost 40% of all patients. ESR may be elevated but doesn't always correlate with disease.

    TYPES OF SLE

    Lupus or SLE has many different types or forms depending upon the organ system involved. Some forms include:

  • 1. Lupus of the skin

  • 2. Lupus of the kidney

  • 3. Lupus of the bone/joints and

  • 4. Lupus of the brain Each of these forms may or may not occur and in many cases, intermingle and correlate with each other as the body preceives an invisible invader.

    TREATMENT

    The goals of treatment are:

    A. Preventative Care:

  • 1. Regular monitoring of the patient, seen by the physician every 3 to 6 months, even if doing well

  • 2. Energy conservation, fatigue is a common complaint

  • 3. Photoprotection: use of sunscreen and avoidance of excess sunshine

  • 4. Infection control: pneumococcal vaccine and yearly flu shots. Considering antibiotics for all procedures, ie.. dental

  • 5. Contraception: avoidance of pregnancy during increased disease activity or while taking immunosuppressive medications

  • MEDICATIONS

    The list of medications used in the treatment of Lupus/SLE are many and can include many that are viewed as chemotherapy in nature. These can include the following:

  • 1. Nonsteroidal anti-inflammatory drugs (NSAIDS) includes current pain relievers: Aleve, Ibuprophen, Aspirin, These drugs are often used in combination with steroids to minimize the dose. Watch out for adverse renal effects.

  • 2. Antimalarials: Planquentil (hydroxychloroquine) is the most common medication used. Effectiveness is seen in skin, joint and mild systemic symptoms. Starting dosage is 400mg/day for 4 weeks then tapering to a maintenance dosage. Some toxicity has been noted in the eyes and might include corneal deposits and retinopathy. Relapse is frequently seen with discontinuation of the medication.

  • 3. Corticosteroids: Many of the symptoms of SLE are the result of inflammation in various tissues of the body. Cortisone, a naturally occurring substance from the adrenal glands and also produced synthetically, has been found to have anti-inflammation properties. Use of these drugs can: reduce the swelling, warmth, tenderness and pain that is associated with the inflammation. One of the most common forms of corticosteroids used in the treament of SLE is Prednisone. Prednisone can be given in many forms: orally, IV, IM or into a joint. Other steroids include: Medrol (methylprednisolone) and Decadron (dexamethhasone). An important aspect of steroid use is that the steroids MUST NOT be stopped abruptly. Instead, a slow reduction of the drug is begun to allow the adrenal glands time to regain their ability to produce natural cortisone again.

  • 4. Immunosuppressive drugs: Also known as cytotoxic drugs. These include: Imuran (azathioprine), Cytoxan (cyclophosphamide) and others. These medications are generally used for 2 reasons: 1. potent drugs which help to reduce disease activity in major organs of the body (kidneys, brain...) 2. may reduce or eliminate the use of steroids in treating SLE. Imuran is less potent and less effective than Cytoxan, but also has fewer side effects. Is usually well tolerated by most patients. Can cause white blood cells, platelets or red blood cells to decrease and increase the risk of developing lymphoma. Cytoxan may cause many side effects, but is also well tolerated by many SLE patients. May cause the same decrease in blood counts as Imuran does. Use of Cytoxan increases the risk of developing malignancies which might include: leukemia, bladder cancer and other tumors. This drug may also cause temporary or permanent sterility in both sexes. And may also predispose you to develop shingles (a painful, blistering skin condition). Hair loss can result from both medications and also the development of infections.

    Medication dosages vary from 125 to 150 mgs. per day orally. Cytoxan can also be given at a much higher dose (1000mg) by IV each month. This is effective for severe kidney damage and also vasculitis.

  • SIDE EFFECTS OF MEDICATIONS

    Side effects of using medications to control SLE vary. The long-term use of steroids can include: cataracts, muscle weakness, premature arteriosclerosis, osteoporosis and bone problems. The relationship between dose, duration and the resulting side effects are usually seen. Higher doses over longer periods of time have the likely result of more side effects than a lower dose over a shorter period of time.

    Side effects noted in use of anti-malarials include: ocular toxicities including corneal deposits and retinopathy.

    Side effects seen in the use of immunosuppressive medications are: increased risks of developing lymphomas and leukemia, bladder cancer and other tumors. Serious decrease in blood counts accounts for many dosage adjustments and frequent monitoring of these blood counts are made during medication therapy. Use of Cytoxan may cause bleeding from the bladder, but this can be prevented with drinking large amounts of water.

  • COMPLICATIONS

    The health status of patients with SLE can change not only with the disease activity but also from the resulting damage to the body from recurrent flareups. This can be seen in the development of deforming joints, end stage renal disease, mental status changes noted in the brain and involvement of the lungs, hearts and vascular system. Other developments can be due to the adverse effects of current treatment, as seen in: anemias, recurrent infections, obesity and bone disorders.

    IS THERE A CURE OUT THERE?

    As we have seen Lupus or SLE affects woman in many different ways. Although there is no current cure available, treatment for the most part, is to reduce the incidence of flare-ups and relapses, reduce the severity of side effects seen with medications and improved health status in general. The prognosis has greatly improved over the years and now patients with Lupus/SLE can expect a 80-90% survival rate over 10 years. This increase is due to the most part in the advancements seen in medical management of the disease...improved medications, more high-tech monitoring of patients and their disease, improved criteria for diagnostic evaluation and overall specialized care to patients with Lupus/SLE.

    Preventative measures and Patient education about the disease can be seen as improving health status and allowing woman to take an active role in the plan of care. Information is widely available now from a number of sources and through the use of the internet, support groups, mailing lists and more have sprung up insuring each patient support and encouragement along the way.

    LINKS TO OTHER SITES ON THE WEB
    Lupus Articles
    The Virtual Hospital: University of Iowa Family Practice..."SLE"
    Pursuit:careers for persons with Disabilities
    CNN Report on Lupus,Feb.19,1997
    Lupus Clinical Overview

    Some useful phone numbers for requesting information on Lupus/SLE are:

  • The Lupus Foundation of America (301) 670-9292 or toll free (800) 558-0121

  • DISCLAIMER: This website is not intended to take the place of a health professional or health practioner. Any ideas discussed here MUST be discussed with your health care provider or health professional in advance.

    © 1997robinhutchens@geocities.com

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