By Dr. John Roberts
Thanks to Jill who asked me to write about lupus. Lupus is the common name for Systemic Lupus Erythematosus. It is an autoimmune disease first described by the physician Rogerius in the 12th Century. There are many opinions regarding the origin of the name. One of the most popular is that many lupus sufferers have a rash on their faces that resembles a wolf’s face. Lupus is Latin for wolf.
Autoimmune diseases are a group of illnesses caused by a person’s immune system attacking various parts of their own bodies. In the case of lupus, the immune system makes antibodies against proteins in the nuclei of cells, the part of the cell where the DNA is found. People who develop lupus are believed to have an underlying genetic predisposition to the disease. There is no single “lupus gene.” Like most genetic diseases, it appears to involve problems with multiple genes as well as interactions with the environment.
Certain environmental factors are felt to trigger damage to cells that results in exposing proteins to the immune system that are normally hidden in the nucleus. Some of the factors causing damage are thought to include ultraviolet light, stress, and medications. Infections, likely by viruses are also hypothesized to be instigators. Drug-induced lupus usually goes away when the offending medication is stopped.
It is estimated between 270,000 and 1.5 million people have lupus in the United States. It is more common in young women who are diagnosed about ten times more often than men. It is usually diagnosed between the ages of 16 and 55 and is more common in African, Hispanic, Asian, and Native American populations. Some of the risk factors for lupus include being related to someone with it (5 to12 percent chance), smoking, sun sensitivity, and post-menopausal estrogen replacement therapy.
Lupus is known as the “great imitator” because it can present with signs and symptoms found in many other diseases. This makes it difficult to diagnose and accounts for an average delay of five years between onset and diagnosis. It is characterized by flare-ups of disease activity followed by remissions.
The most common signs and symptoms of lupus include joint pain (especially the hands, wrists and knees), fever, malaise, rashes, muscle pain, fatigue, and weight loss. Some women report flares after ovulation and improvement with menstruation.
Since lupus involves an immune response, it can affect every organ system in the body. The most commonly affected include the heart, joints, skin, lungs, blood vessels, kidneys, and nervous system. Joint involvement is present in about 90 percent of patients. The typical lupus rash is found on the cheeks and nose but can also present with scaling lesions elsewhere. Some people develop severe inflammation of the heart, kidneys or lungs.
Diagnosing lupus can be very difficult. A patient has to have at least four of eleven symptom criteria either together or over time. Each of the eleven criteria has varying specificity (if present the disease is likely) and sensitivity (if absent the disease is unlikely) for diagnosing lupus.
There are also laboratory tests that can help confirm the diagnosis. The most common is the ANA or Antinuclear Antibody test. If this test is negative, the likelihood of lupus is very low. If it is positive, confirmatory tests that look for antibodies to proteins in the nucleus are often done.
There is no cure for lupus, so the goal of treatment is to prevent flares and shorten the duration and severity of symptoms. There are many types of medications used. First line drugs are non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen and Aleve. If those fail, or more severe symptoms develop, stronger medications can be used to suppress the immune system.
There are also lifestyle changes that can reduce symptoms or limit flares. Patients should avoid direct sunlight and wear a high SPF sunscreen. Smokers should quit and those who are obese should lose weight to reduce stress on their joints.
The prognosis for lupus has improved dramatically. In the 1950s almost all patients died within five years of diagnosis. Patients can now expect to live five years 95 percent of the time. Most who die of lupus do so from infections from damaged organs or immune suppression from medications.