In the medical world, there are some illnesses that are so all-encompassing in their development and effects that they become a model for understanding the basic nature of health’s deterioration into illness. Lupus, more properly called Systemic Lupus Erythematosus, is one such disease. Mentors in medical schools quip “if you know lupus, you know medicine!” Though uncommon, it is rarely far from a physician’s mind, for it is “the great imitator” and can cause virtually any symptom! Headache, fatigue, rash, seizures, hair loss, muscle weakness, palpitations, abdominal pain—any of these COULD be due to lupus, though seldom is. It is one of the most difficult tasks in healthcare: to focus on the common – but never forget the strange. Testing for the possibility of this rather exotic disease is infrequently done during any initial evaluation, but routinely performed in working up a persistent “mystery” condition.
Lupus is an autoimmune disorder. The underlying error that ultimately creates this sometimes deadly condition stems from a misdirected immune system, (similar to rheumatoid arthritis). When working properly, the powerful immune mechanism of our body is able to hone in on and destroy foreign/infecting entities. But in lupus, this system is mistakenly triggered to produce destructive proteins—antibodies—against one’s own body parts. Depending on which organ the antibodies erroneously attack determines the symptoms. For instance, certain antibodies destroy kidney cells and cause kidney failure; others work against bone marrow cells and lead to anemia or a serious bleeding disorder due to low platelets. The most common manifestation of lupus is caused by antibodies against components of the skin, such as the classic facial rash involving the bridge of the nose and both cheeks—the so-named butterfly rash. Another common skin symptom—circular bald spots— results from antibodies attacking the scalp. But it is severe joint pain caused by antibodies against the “soft tissues” of the joints, the tendons and ligaments that initially bring most lupus patients to a doctor’s office.
The diagnosis of systemic lupus erythematosus is complicated. There are readily available blood tests that check for the presence of auto-antibodies—the most common one being ANA antibody. But plenty of people have rashes, joint pain and a positive blood test and still don’t have lupus! Scientists agree that there are countless different manifestations of this illness and that to be considered true lupus, a patient must have many symptoms or findings, such as abnormal blood tests, skin findings and at least one instance of organ failure. A primary care physician can put forth the diagnosis of lupus, but it invariably needs to be confirmed and managed by a specialist of auto-immune disorders, the rheumatologist. Lupus occurs most commonly in women of child-bearing age, and until recent times had a poor prognosis. Now, fortunately, the outlook is much better, with over 75 percent of patients alive and well 20 years after diagnosis. But though it can now be controlled, it is virtually never cured and relapses do occur.
As bad as lupus is, it can be tamed, for now there are treatments able to reign in a misdirected immune system. Initially, steroids, which can nonspecifically squash immune activity, such as prednisone, are used. These drugs can make symptoms abate quickly, but their long term side effects of diabetes, osteoporosis, obesity and frequent infections make rheumatologists work to get their patients on an alternate regimen as soon as possible. Chemotherapy agents are now the main medicines used to achieve and maintain lupus remissions. Though they would be deadly if given at the usual anticancer doses on the extended basis needed in lupus patients, they are very effective and well-tolerated in much smaller quantities. Once remission has been achieved, treatment can often be pared way down or even temporarily stopped. These remissions are rarely permanent, though, so continued specialist follow-up is needed. Hopefully, further study will allow scientists to identify the presumed genetic flaw that is the cause of this severe body-wide disease, so they may ultimately correct things before this unfortunate condition, systemic lupus erythematosus, arises.