Lupus: The Unknown Common Disease

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LUPUS: THE UNKNOWN COMMON DISEASE

The wolf that took down author Flannery O'Connor in her prime today yields to hope and promise as science unlocks the mysteries of the immune system.

Lupus (Latin for wolf) erythematosus (Greek for reddish) was the name 19th century physicians gave to a mysterious disease that often caused a rash on the face that resembled the bite of a wolf. Systemic lupus erythematosus (SLE), commonly shortened to lupus, is an autoimmune disease in which the immune system turns inward harming the body's own tissues.

Very simply, lupus is a case of the body not recognizing itself. For some as yet unknown reason, the immune system goes awry and begins manufacturing substances called antibodies against its own tissues, causing painful and sometimes dangerous inflammation.

Lupus affects one of every 2,000 Americans, or approximately 500,000 people. Lupus is more common than cystic fibrosis, multiple sclerosis, muscular dystrophy, leukemia and cerebral palsy, but suffers more than any of these diseases from lack of awareness. Unfortunately, many who know the term associate it with AIDS or some other infectious disease; to be sure, lupus is not contagious.

Lupus is as voracious as it is mysterious. It can ravage not just one, but a host of sites within the body, producing a multitude of symptoms. Desperate fatigue, migraines, skin rashes, fever and joint pain are just a few of the symptoms that wax and wane, either in unison or succession. Symptoms often mimic other diseases, frustrating patient and physician alike and delaying diagnosis. Some patients think they must be imagining such varied and seemingly unrelated symptoms; worse yet, their all-encompassing symptoms violate traditional medical thinking and can mystify even the best of physicians.

WHO GETS LUPUS?

Lupus is diagnosed in patients from childhood into the ninth decade of life. Most commonly, this complex disease strikes women during their childbearing years. Overall, females are affected nine times more commonly than men. In childbearing years, this ratio rises to fifteen to one. Lupus also shows racial preference, occurring three times more commonly in black women than white women.

WHAT CAUSES LUPUS?

Lupus is one of the few diseases for which the cause remains unknown, but research on the immune system has offered a greater understanding about the origins of lupus. "In very recent years we have discovered that there are at least six genes responsible for lupus, but we don't know what genes they are," explains Arthur Krieg, M.D.,former lupus researcher at the National Institute of Arthritis and Musculoskeletal and Skin Diseases, and currently Assistant Professor of Medicine at the University of Iowa. "Some people may have one gene that predisposes them to lupus, others may have all existing lupus genes. But genetics are not the only determining factor in whether or not a person displays lupus. We know that various environmental factors influence who will show active signs of the disease. For example, ultraviolet radiation (the sun and some fluorescent lights), certain foods, drugs, and chemicals, such as hairdyes, may activate disease in those who are predisposed because of genetics." Even though genetics are involved in lupus, Dr. Krieg explains, there is only about a four percent chance of passing the disease on to offspring.

Six hormones offer at least a partial explanation of why women are more commonly affected and why the disease can be more active during pregnancy and immediately after childbirth. Dr. Krieg says that the role of sex hormones in lupus is studied in several strains of mice that get the same syndrome of lupus that occurs in humans. As in humans, female mice are affected with lupus more than male mice. When the females are given male sex hormones (androgens) the incidence of lupus among them is greatly reduced. "Conversely, if you give male mice female sex hormones and/or castrate them, they get lupus much more frequently." Interestingly, research on men with lupus reveals that some are hormonally indistinguishable from women.

Even though the hormones of pregnancy and menses and the tremendous hormonal changes following pregnancy are associated with greater disease activity, the effect of anti-estrogen or synthetic androgen treatment on human SLE have been at best inconclusive, at worst disappointing.

WHAT HAPPENS INSIDE THE BODY?

Normally, the immune system protects against such foreign invaders as bacteria, viruses and fungi. In lupus, however, antibodies are formed against the body's own tissue. These autoantibodies, as they are called, cause a complex sequence of immune responses. The autoantibodies combine with other immune substances to form immune complexes. Immune complexes become lodged in selective tissues, frequently connective tissue, causing inflammation. Connective tissue is found through the body binding cells and tissues together; examples are tissues around joints and those lining the heart, lung, and abdominal cavity. The immune complexes can also deposit in the kidneys, interfering with kidney function. Tissues are also damaged as the body attempts to destroy the immune complexes, a process which releases harmful chemicals.

WHAT ARE THE MOST COMMON SYMPTOMS?

The table on page 36 lists the clinical features of patients with lupus, and the percentage of patients in which they occur. Disease activity, often called a flare, may cause one or several symptoms in a patient. Then, the disease may become quiet, or go into remission, for a period of weeks to months to years. When the disease flares again, symptoms in a patient may be entirely different from the last flare. Or, they may be the same as those previously experienced, or they may be a mixture of new and old. The most common symptoms, according to Peter H. Schur, M.D., Professor of Medicine at the Harvard Medical School and Director of Lupus Research at Brigham and Women's Hospital in Boston, are fever unrelated to infection, weight loss, fatigue, joint pain, facial rash, kidney trouble, and chest pain. Some of these symptoms are described in greater detail:

Fatigue, a tiredness that defies description, is a persistent and frequent problem. Henrietta Aladjem, a founder of the Lupus Foundation of America, and the author of several books on lupus, recalls the fatigue she experienced:

"Before I got sick I was full of life; I loved to play tennis, ski, hike in the mountains in all kinds of weather. Then everything changed. My whole body ached and I became tired and lifeless. The fatigue was the symptom that bothered me the most. It was not an ordinary fatigue. It was a drained feeling, a lethargy that did not improve with rest and relaxation; it absorbed my whole person, physically and emotionally. I was so exhausted I could barely lift my arm to attend to my personal needs. I was pathetic. I would sit down in a chair, and when I wanted to get up I could not. I was so tired I felt paralyzed."

Joint pain plagues approximately 90 percent of patients. The fingers, hands, wrists, knees, ankles and elbows are most commonly affected. This joint pain is called arthralgia. Arthritis, which is inflammation causing deformity and x-ray changes in a joint, is less common in lupus patients.

Skin manifestations are also very common, occurring in about three quarters of lupus patients. The classic butterfly, or malar, rash is a red flat or raised rash occurring over the cheeks and bridge of the nose in a butterfly pattern; sometimes the chin and ears are affected.

Other skin symptoms include loss of hair, painless oral and nasal ulcers and photosensitivity. About 40 percent of lupus patients are very sensitive to ultraviolet (UV) light, a type of light waves found in sunlight and many types of fluorescent light; some even to the light emitted from photocopy machines. In many cases, exposure to UV light will cause a characteristic rash and may activate disease in other body parts. Curiously, systemic symptoms may flare after UV exposure even though this exposure does not cause a rash. Researchers think UV light activates this disease by exerting some effect on the genetic material.

Kidney disease occurs in approximately half of lupus patients. Disease in the kidneys ranges from mild to severe, and can lead to kidney failure. Kidney failure remains the leading cause of death in lupus patients, but careful monitoring and better treatment options have improved this.

Other common symptoms are serositis, which is an inflammation of the serosa, or lining, of the heart (pericarditis), lungs (pleurisy) and/or abdomen (peritonitis), all of which are very painful. Various blood abnormalities, including anemia, low white count and low platelet count, also occur. Neurological symptoms include seizures, migraine headaches, stroke and psychiatric problems. Psychiatric problems caused by neurological inflammation range from behavior change and confusion to hallucinations and psychosis. Dr. Schur emphasizes the difficulty in distinguishing between "the natural fears and anxieties that accompany chronic illness (and symptoms) of organic neurological disturbances." This is also complicated by behavior changes caused by steroid medications.

Other disease activity can be found in the eyes, liver, heart, lungs and gastrointestinal tract.

WHAT CAUSES A FLARE?

Lupus is a disease that waxes and wanes from its onset throughout a person's lifetime. Certain factors, such as infections, ultraviolet light, various medications, pregnancy, and emotional stress may precipitate a flare. The cause of some flares, however, is unclear. Lupus patients learn to recognize situations and activities that have caused them to flare in the past and try to avoid them. For example, photosensitive patients must stay out of the sun between 9 a.m. and 4 p.m., longer if very sensitive or in areas nearer to the equator. Infections are always a problem, and patients and doctors generally learn how to treat an infection to prevent a flare of disease activity.

TREATMENT

In the 1950s, half of lupus patients died within five years of their diagnosis. Today, more than 90 percent of lupus patients live a minimum of 10 years after diagnosis; many have a normal life span. Most deaths from lupus, in fact, are preventable with early and appropriate treatment.

There are no cures for the underlying processes of SLE, but several medications are available to treat symptoms and calm disease activity. Treatment is multifaceted: The ultimate goal is to prevent disease activity. But when inflammation occurs, prompt and appropriate treatment should reduce it and prevent organ damage.

Aspirin and non-steroidal anti-inflammatory drugs are commonly used for joint pain. Antimalarial medications are used with great success in some patients to control skin rashes and joint pain. According to Dr. Krieg, recent research indicates that the antimalarial hydroxychloroquine may actually correct one of the immune defects which causes lupus.

Adrenal corticosteroids and immunosuppressive agents (cancer drugs) are used for more severe disease activity. Dr. Krieg also indicates that treatments on the horizon may affect the cells that cause the automimmune response. "Most importantly," says Dr. Krieg, "treatment must be individualized for each patient. Just as no two patients exhibit the same disease characteristics, no two patients will respond to the same therapy. Sometimes one combination of medications works on one patient, another combination on another patient. Patients and physicians must work together carefully to determine the best treatment for the stage of the disease."

Rest is essential in the treatment of lupus. Reassurance is vital. Says Dr. Schur in Henrietta Aladjem's book, In Search of the Sun:

"The patient, fearing the unknown or having heard the worst, must be reassured that a majority of patients have mild to moderate disease and that with proper management many of the more serious complications of organ damage can now be prevented and/or controlled."

CONQUERING LUPUS: AWARENESS, UNDERSTANDING, PERSONAL STRENGTH

Lupus does not have to preempt a productive, satisfying life. A well educated patient must form a partnership with a compassionate physician to combat this powerful disease. A patient must strive to understand the disease and what to expect from it. and then learn what to report to the physician on regularly scheduled checkups. Checkups are essential during times of disease activity and quiescence alike. A lupus patient needs to understand that although symptoms are painful and debilitating, and at time relentless, many are not life-threatening. It is in recognizing and understanding the potential harm of disease activity that a patient can conquer the worst; it's like a football team meeting its opponent head-on after studying videos of previous games and knowing where and how hard they tend to hit. Mrs. Aladjem, in remission for over twenty years, says in response to people who ask her what she does to stay well: "I am a perfect example of Sir William Osler's aphorism: `If you want to live a long life, get a chronic disease and learn how to take care of it.'"

American Council on Science and Health, Inc.

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By Kristine Napier

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