Guest Post by Donald E. Thomas, Jr., M.D., FACP, FACR
“To know lupus is to know medicine” is a common saying repeated by attending physicians to their medical students and residents. For the medical student and the young physician, this can create some anxiety and can be quite intimidating. Even experienced physicians can find it a great challenge to adequately care for the person who has lupus, especially the form of the disease called systemic lupus erythematosus. How can you possibly take care of a disease where you have to know so much? Imagine being the patient confronted with a disease that is so complicated that it can perplex the best of physicians.
The word “lupus,” which means “wolf” in Latin, has been used to describe diseases since the Middle Ages. The wolf of medieval Europe must have been seen as a veracious animal capable of causing significant harm to humans. Diseases that caused damage to the skin reminiscent of wolf bites were called “lupus” in order to describe what people could see and relate it to something in their own environment. The very first description of lupus was a ninth-century account of the bishop of Leodicum (present day Liège in Belgium), which described how the bishop was afflicted by a disease nibbling away at his flesh and internal organs. The writer even stated that “lupus” was a common name for a disease in those days. The involvement of the skin and internal organs certainly can lead us to wonder if the bishop had a variant of the disease known today as systemic lupus erythematosus. But, because of the absence of any true medical knowledge in those days, the terms lupus could have been used to refer to various diseases, such as cancers, infections (like tuberculosis), or other systemic disorders (including sarcoidosis).
The color print shown here depicts the curing of the bishop of his lupus by Saints Martin and Brice after doctors’ applications of eviscerated chickens to his wounds did not succeed. Therefore, this account contains the very first mentions of early failed treatments for lupus, as well as evidence of the disease’s first “cure.”
Physicians consider lupus the prototypical autoimmune disease because it involves all parts of the immune system and can attack all parts of the body. It is genetic in origin; over thirty well-described genes cause it to occur, yet most people who are born with the genes never develop it. The more genes that someone inherits, the more likely it is that they will develop the disease. Environmental influences, called triggers, are also required to initiate its development. These triggers are diverse and include viral infections, excessive sun exposure, smoking cigarettes, eating alfalfa sprouts, and even taking herbal supplements used to “boost” the immune system such as echinacea. The autoimmune genes responsible for causing lupus are also responsible for causing a wide array of other autoimmune disorders such as rheumatoid arthritis, scleroderma, Sjögren’s syndrome, and autoimmune thyroid disease. Doctors are discovering that certain genes can predispose a person to certain disease manifestations, so it is not surprising that the number of potential combinations of these genes could cause a wide array of possible disease manifestations.
One medical article (Kolstoe J, Value-Based Care in Rheumatology. OCT 2013;2(5):10) calculated that inheriting just four of these genes results in 37,405 potential different gene combinations. This can help explain why every person who has lupus is different. One person can have kidney disease and joint pains, another could have recurrent strokes and ulcers in the mouth, while another could have very mild disease with a slightly low white blood cell count and feel perfectly fine.
Lupus can be difficult to diagnose. Surveys suggest that patients on average have symptoms of their disorder for three years before a correct diagnosis is made, and have often see numerous physicians during this period. It is not uncommon for the patient to be told they have a simple viral infection, or even that there is nothing wrong with them at all. It has to be very frustrating to many patients and you can certainly see how it could be easy to distrust the medical community during this process.
The treatment of lupus is necessarily complex. Medications are required to prevent the immune system from attacking the body, while other medicines are used to prevent side effects from those immunomodulating medications. Medications that may work wonders in one person may do nothing in someone else with similar manifestations. Therefore, the treating physician often relies upon a “trial and error” approach with various regimens before discovering an effective management plan. Patients must learn that using sunscreen daily and taking vitamin D are essential treatments. In addition, the treatment of lupus is not confined to taking medications. People who have lupus must also learn how to avoid all the things in their environment that can make their disease worse.
The person who has lupus must see a doctor every one to three months in order to get lab tests and undergo a physical examination to ensure that their lupus is not attacking various parts of their body. The overall costs (monetary, time-wise, physical, and psychological) can easily become overwhelming.
Most people who developed lupus before the first treatments became available in the 1950s died very shortly after their diagnosis was made. Today, most people who have lupus can expect to live a long, normal life span if they are compliant with their treatment plan. This statement is not meant to diminish the fact that lupus is still a deadly disease. It kills many of its victims every year, while others are left with horrible disabilities.
The good news is that there are many things that people with lupus can do in order to control their disorder better. The purpose of my upcoming book, The Lupus Encyclopedia: A Comprehensive Guide for Patients and Families, is to put all of this information into one easy-to-read source. My book goes into detail about the manifestations of the disease and the many potential complications that may occur as a consequence of the medicines, such as osteoporosis and osteonecrosis. I also cover the negative consequences, including fibromyalgia and depression, that may occur when the mind and body has to deal with chronic inflammation. Since the treatment of lupus can be daunting, a simplified list of “do’s and don’ts” called “The Lupus Secrets” is presented in one chapter in order to allow someone to start to quickly learn the right things to do while the other chapters go into greater detail about the proper execution of these “secrets.” Tidbits related to the history of lupus are interspersed in order to add some historical context to a book packed with medical and scientific information.
My primary purpose was to provide an all-inclusive guide for people who have lupus and try to demystify many of its complex problems and treatments. It is meant to be very practical at the same time for every aspect of lupus care, to include what to truly expect from the medicines rather than just relying on a generic laundry list provided by a pharmacy or the FDA-required and unjustly scary side effects they may see on television ads about their medications.
One of the most exciting aspects of my book is how I am incorporating social media. I opened a Facebook page and a Twitter account in order to provide a running update on new medical information about lupus so that patients could follow and add to what they read in the book. Readers can also reach me at email@example.com. This allows people to give me feedback on what they would like to see in future editions of the book and encourages people to point out potential errors in the manuscript so I can correct them in the book and provide people with as much knowledge about their disorder as possible. Medical advice cannot exist without opposing viewpoints, so this will also allow those with different views to express their opinions on controversial topics.
My Facebook page has truly exploded into a place where people with lupus from around the world interact with me as well as with each other. I am already learning about additional problems that people with lupus face and the things that they want to know more about. This interaction is opening up my eyes as a physician regarding unforeseen questions and problems in the life of a lupus patient. Too often, lupus wrecks lives and causes its victims to feel powerless. I feel strongly that the person with lupus who uses this book will in their own way “know lupus” and “know medicine,” allowing them to become empowered again and gain control over lupus and their own lives.
Donald E. Thomas, Jr., M.D., FACP, FACR, is an assistant professor of medicine at the Uniformed Services University of the Health Sciences and teaches rheumatology at the Walter Reed National Military Medical Center. He is a practicing physician and currently serves as a member of the Medical-Scientific Advisory Council of the Lupus Foundation, DC/MD/VA chapter. His book The Lupus Encyclopedia: A Comprehensive Guide for Patients and Families is now available.