“I’m not sure, but I think you may have lupus.” These are the uncertain words lupus patients often first hear of their disease. Systemic lupus erythematosus is an “autoimmune disease”—a disease in which the immune system attacks one or more of the body’s own tissues instead of defending itself from invading bacteria and viruses as it is supposed to do.
The immune system has two principal types of cells, B cells and T cells, to protect us against invading germs. B cells make proteins called “antibodies,” which are the bullets that kill these germs. T cells control the B cells and also kill virus-infected cells themselves. In lupus, for some unknown reason, the B cells and T cells become confused. The T cells direct the B cells to make “autoantibodies,” which are antibodies directed at one’s own cells. In addition, the T cells become “autoimmune,” and kill healthy cells.
The resulting autoimmune attack can cause a remarkable variety of symptoms, depending on which tissues are involved. For example, lupus frequently attacks the skin, producing rash; the joints, causing arthritis; the blood, leading to anemia (low red blood cell count) or decreased numbers of platelets (particles in blood which help blood to clot); or the lining of the lung, resulting in pleurisy.
This overview discusses mainly the systemic form of lupus. Another form of lupus is discoid lupus, which is also an autoimmune disease. However, 95% of the time, discoid lupus remains restricted to the skin and does not involve internal organs. A third form of lupus is called subacute cutaneous lupus erythematosus. Like discoid lupus, most patients with subacute cutaneous lupus erythematosus do not have systemic lupus erythematosus; their autoimmune disease generally remains restricted to the skin. These closely related conditions are forms of lupus and share some common features. However, in general in this overview, we will use the term “lupus” to refer to SLE, the systemic form of the disease.
Because of the highly variable and often confusing symptoms, it is unusual for patients with symptoms of lupus to be correctly diagnosed by the first physician they see. Although it is more common than multiple sclerosis, lupus often is not considered initially by the physician evaluating a patient. In some lupus patients, the diagnosis is obvious, and the treatment prompt and effective. Potentially life-threatening manifestations of lupus (such as kidney disease) are generally very clear, even though it may not be apparent that the lupus is the cause. In other lupus patients, non-specific symptoms such as fatigue and achiness may predominate, making the diagnosis more difficult.
In most lupus patients, the diagnosis is not at first obvious but gradually becomes apparent. The average lupus patient waits for about two years from the time symptoms appear until they are finally told with confidence that they have lupus. Besides feeling extremely frustrated and wondering if they are really imagining some of their symptoms, such patients may go from one doctor to another with the hope of finding someone who knows what they have and how to treat it. Physicians who do not recognize lupus sometimes think that patients have a psychosomatic disorder and refer them for psychiatric therapy.
Patients may have such unhappy encounters with physicians that they lose faith in the medical profession. We hope that as lupus becomes better known by doctors and lay people, such experiences will become less common.
History of Lupus Research
Several centuries BC, Hippocrates described a skin rash that may have been a form of lupus. In centuries since, the term “lupus” (from the Latin for “wolf”) was initially used to describe a disease marked by ulcerating skin lesions (which looked like wolf bites), typically on the face. Today, such patients are diagnosed with discoid lupus. The association of lupus with the now well-known “butterfly” facial rash was described in the mid-1800’s. By the late 1800’s, several European physicians had realized that lupus could affect the internal organs as well as the skin. A Viennese physician, Moritz Kaposi, went so far as to suggest that there were two types of lupus: one that primarily affected the skin and another that could cause a fatal systemic illness. In the United States, it was Sir William Osler at the Johns Hopkins Hospital who first reported the systemic form of lupus.
Over the following decades, physicians around the world carefully documented the pathologic (involving or resulting from disease) changes of lupus but had no accepted treatments to offer nor any clear idea of what caused lupus. The first useful test for diagnosing lupus was reported in 1948 by Drs. Hargraves, Richmond and Morton at the Mayo Clinic. They observed a peculiar type of cell they termed the “L.E.” cell (for lupus erythematosus) which was frequently present in the blood of lupus patients. The realization that these cells resulted from cellular destruction led to the suspicion that lupus was an autoimmune disease. This suspicion was confirmed by a report in 1957 that anti-DNA antibodies were present in the blood of lupus patients. Since then, our understanding of lupus and our ability to treat this disease have advanced considerably.
Dr. Arthur M. Krieg is recognized for his research which established that the immune system recognized bacteria and viruses not only by telltale features of their protein clots, as scientists have long known, but by simple yet distinct patterns in the microbe’s DNA. Now his further research has shown that bacterial DNA can activate the immune system, suggesting a possible explanation for the fact that infections often seem to trigger lupus flares. The immune activation by germ DNA is blocked by anti-malarials which may provide a clue to the beneficial side effect of anti-malarials in treating lupus.
Reprinted with permission from Lupus Awareness, the newsletter of the Iowa Chapter, LFA.