Lupus - Systemic Lupus Erythematosus

Lupus - Systemic Lupus Erythematosus (SLE)

Systemic lupus erythematosus or SLE is an autoimmune disease of still unknown cause. Women of childbearing age develop it much more than men. Its symptoms imitate those of other diseases, especially fatigue, hence it is different to diagnose. There are 4 known types. Approximately 1.5- 2 million women develop SLE. There also appears to be a genetic component to it. There is still no known cure to it but more and more drugs are being developed to at least control its symptoms. Lupus patients require special attention from his family and community, which must make community resources available for their coping. Some of them get deeply depressed or unreasonably tired. Worthless worrying further deprive them of sleep and wake up more tired in the morning.

Introduction

Autoimmune diseases are especially life-threatening conditions, which everyone needs to know about and be guarded against. One such disease is systemic lupus erythematosus or SLE. Its symptoms mimic other disorders and can spread into the body systems before anything can be done. Although survival has improved in the last 3 decades, there is as yet no cure for it. Lupus sufferers present physical and psychosocial complications, which all require attention and expert remedy or handling.

Body

Medical Characteristics, Types

Systemic lupus erythematosus or SLE is an autoimmune disorder wherein the immune system turns against the body and destroys healthy tissue (NWHRC, 2008). Normal immune system fights against and protects the body from bacteria and viruses. In SLE, this is reversed. Healthy tissues include skin, joints, kidneys, heart and lungs. SLE can create life-threatening problems or chronic symptoms, which reduce the quality of life. Chronic low-grade symptoms include fatigue and muscle aches. It affects many body systems at the same time, that is why it is disabling and life-threatening (NWHRC).

SLE involves only the skin and joints in some people but also the lungs, blood and other organs or tissues in others (NWHRC, 2008). Not all the symptoms are suffered by all persons with the disease. There may be remission periods of few or no symptoms and periods of "flares," when the disease becomes active. Another type is discoid lupus erythematosus or DLE. In this condition, parts of the body exposed to the sun develop lesions of very red, raised and hard bumps or plaques. Among these may be overgrown scaly tissues, plugged hair follicles and very wide blood vessels. Older lesions may include atrophic scarring and dyspigmentation. Atropic scarring is thinning of healing skin. Dyspigmentation is loss of color of the skin. Some sufferers may have DLE without SLE. In about 10% of cases, DLE progresses to more severe SLE. Lesions occur above and below the neck. A third kind is drug-induced lupus. The most common drug is procainamide, which is prescribed for heart rhythm problems, hydralazine for high blood pressure, and isoniazid for tuberculosis. This does not affect the kidneys or central nervous system. It also improves when the drug inducing it is stopped. On the other hand, neonatal lupus develops in newborns when auto-antibodies from the mother pass on to her baby. These are specifically anti-Ro/SSA or anti-La/SSB. They affect the skin, heart and blood of the infant. An uncomplicated rash within the first many weeks is the most common symptom, which persists to six months, before vanishing. These fetuses also often develop a congenital heart block (NWHRC).

Drug-induced lupus develops from the use of minocycline intended for acne and hydralazine intended for heart failure (Walsh, 2008). Minocycline-induced lupus was first recognized in early 1990s. Since then, the World Health Organization has recorded 250 cases, mostly women at a 5:1 ratio. Major signs are fever, morning stiffness, myalgias, polyarhralgias and symmetric arthritis. And 60% of those afflicted also complain of large vessel vasculitis and the presence of anti-neutrophil cytoplasmic antibodies. Dr. Andrew G. Franks, Jr. commented that the use of minocycline enhances the formation of lupus-like syndrome 8.5 times. This led health professionals to substitute doxycycline for minocycline for the treatment of ane and rosacea. Other drugs implicated with lupus include hydralazine, procainamide, isoniazid, quinidine, and phenytoin and almost 100 others. Symptoms of classic drug-induced lupus resemble those of influenza. Most of those afflicted are ANA and antihistone antibody-positive (Walsh).

The third type of drug-induced lupus is called sub-acute cutaneous lupus erythemaosus or SLCE (Walsh, 2008). It is associated with various agents, such as thiazide diuretics, antifungals, calcium channel blockers, and ACE inhibitors. Some reports link SLCE with statins, leflunomide and tumornecrosis factor inhibitors. SCLE is difficult to determine. Suspected lesions are often mixed with papulosquamos of annular lesions. These can be mistaken for erythema multiforme or toxic epidermal necrolysis with dis-adhesion of the epidermal layer and sloughing of the skin, according to Dr. Franks. Withdrawing the drug leads to remission in 95% of patients with additional therapy. On the whole, nothing can as yet explain why these drugs induce lupus or produce clinical and laboratory manifestations. Some guesses have been advanced, however. The drugs can act as haptens or antigens, which elicit an immune response. Or these drugs act as immune system modulators, which enhance the development of self-directed responses (Walsh).

Incidence

The Lupus Foundation of America reported that 1.5 to 2 million people in the United States are afflicted with some form of this disease. It occurs 10 times more in adult women than among men (NWHRC, 2008). It was found to occur more among African-Americans, American Indians and Asian than Caucasians. It is also diagnosed most often among women of childbearing years. The risk is higher among those with a family member with the disease. A health professional is not likely to conduct a test for SLE unless a suspected member develops the symptoms. There is no known cure for SLE, but treatments are available to reduce symptoms and their effects (NWHRC).

The genetic predisposition to lupus is complicated in that it involves several genes, which interact and determine the person's risk (NWHRC, 2008). Small inherited differences among body proteins may cause these to under-react or over-react as part of the immune system. Any of these small differences is enough to create a health problem. Those who develop SLE are believed to inherit these small differences, which differ from patient to patient. Different immune systems enabled the human species to survive. If all of them were identical, the race should have perished (NWHRC).

Complications

SLE increases a woman's risk for cardiovascular disease more than two times (Kubetin, 2008). This was the conclusion of the recently concluded Nurses Health Study. The study was conducted on more than 120,000 women, aged 30 and 55, in 1976. They were free of both cardiovascular disease and SLE when they enrolled for the study. After 28 years of follow-up, 148 of them developed SLE. Most of them were diagnosed at an average age of 56. Those who developed SLE mostly developed hypertension, diabetes and cardiovascular disease (Kubetin).

Diagnosis

Doctors recommend early detection to reduce the chance of organ damage and other complications (Meadows, 2005). The diagnosis is made on the basis of symptoms, medical history and exam, blood and urine tests. Lupus can lower blood counts and affect kidneys and bring protein and blood in the urine. Biopsies of the skin or kidneys may be done. Removal and examination of tissue may be performed to find signs of autoimmune disease. The anti-nuclear antibody or ANA test is commonly used to look for auto-antibodies, which react against the nucleus. Most lupus sufferers have elevated ANA. A positive ANA is, however, not sufficient to confirm lupus, as 20% of healthy women may have a positive ANA (Meadows).

Treatments

Protein Blockers

Findings of a new research offered to explain the rationale behind the development of SLE (Podiatry Now, 2008). Immune cells, which die in a normal person's body, accumulate in a person afflicted with lupus. This condition contributes to the development of the disease. The research was performed on 14 lupus patients and 14 healthy persons. The findings also discovered that lupus patients had more immune cells, which carry proteins, which prompt these cells to extend life beyond normal limits. The signals for these cells to die are turned off and these cells remain and accumulate in the body. As a consequence, diseases present are allowed to persist and spread (Podiatry Now).

Team members of the research, which was conducted at the Saint Louis University, said they would conduct more pertinent studies (Podiatry Now, 2008). These would be on therapies, which could block the action of these proteins, by imitating their action but instructing immune cells that it was time to die. Dr. Louis of the University said they would target those cells, which keep immune cells alive beyond their time. He said that such treatment could induce remission. His treatment would enable normal cells, which do not want to attack the body, to function properly. It would kill cells, which persist to perpetuate the disease. The intended consequence would be for the body to fight infection and for the person to live normally (Podiatry Now).

CellCept drug for the treatment of kidney complications could be a boon to lupus patients (Chang, 2005). A small study showed that the drug delivered better results than standard chemotherapy, which could cause infertility and other medical problems. A recent experiment compared the effects of CellCept and the older treatment, cyclophosphamide, in patients for 6 months. Those taking CellCept reported fewer side effects. The researchers were led by Dr. Ellen Ginzler of the SUNY Downstate Medical Center. The disease develops mostly in women of childbearing age. The immune system attacks its own organs and tissue. The cause is still unknown. But in a third of patients, the most common symptom is inflammation of the kidney. This can, in turn, lead to kidney failure (Chang).

Chemotherapy has been the standard treatment for lupus for the past 30 or more years (Chang, 2005). But it produces unpleasant side effects, which include hair loss, nausea and infertility. These side effects discourage many patients to discontinue treatment. CellCept is an FDA-approved drug, manufactured by Hoffman-La Roche, Inc. It is used primarily to prevent organ rejection in transplant patients. But it is recommended to lupus patients who cannot endure chemotherapy. A study was conducted on 140 lupus patients who received daily doses either of CellCept or cyclophosphemide intravenously. Findings showed that 23% of those on CellCept had complete remission and 30% had partial remission. These figures were compared with 25% of those who received chemotherapy. Those patients who received the drug also had fewer infections and hospitalizations, although they experienced more bouts of diarrhea. Dr. W. Joseph McCune of the University of Michigan Medical Center commented that it would be reasonable for doctors to prescribe the drug to patients who were concerned about fertility (Chang).