Lupus Digest Basic Facts
Copyright © 2012 A. G. Moore, Westbury, NY
Web site: thesearethefacesoflupus.com
Smashwords Edition
This ebook is licensed for your personal enjoyment only. This ebook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each recipient. If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy. Thank you for respecting the hard work of this author.Disclaimer: Nothing in this book is meant to prescribe or endorse any treatment or medication. The opinions offered are those of the author and carry no kind of warranty as to accuracy or effectiveness. No source, website or otherwise, cited in this publication is endorsed by the author.
Cover Picture: The Helix Nebula spewing ultraviolet radiation in outer space; on earth, UV radiation is harmful to people who have lupus. The picture is from NASA and is in the public domain.

Books by A. G. Moore:
A Lupus Handbook: These Are the Faces of Lupus
A Mask for Every Face
Prelude to a Vampire
Ernesto Learns to Love His Nose (for children)
Table of Contents
Use The Life You Have
Lupus-What Is It?
Lupus Symptoms
Possible Causes
Lupus Treatment
Use The Life You Have
I am not a doctor or scientist--I am a patient. My experience as a patient has taught me much. It has taught me that the more I know and the more I understand about lupus, the more likely I am to live productively with this disease.
All people who have a serious illness should recognize that skilled medical care is essential to their health. What may not be clear is that patients have a significant role to play in insuring they receive the proper care. Every treatment decision entails the weighing of risks and benefits. Personal values influence the course chosen. Always, in this process, patients must keep in mind that they are the ones who will benefit from--or suffer--the consequences of a treatment plan. So it makes sense that patients assume some responsibility for determining treatment options.
This is why I began to read, and to learn, about lupus. The impulse to write came later.
When the first symptoms of lupus intruded into my life, I was unfamiliar with the disease. I could tell by the doctor's demeanor at that first visit this was a serious illness. Realizing that I might be facing a formidable enemy, I decided never to go back to the doctor again; I simply refused to deal with the problem.
It's been a long time since it was first suggested I might have lupus. My early ignorance and denial were not useful in controlling the course of the disease. Doctors I consulted may have been well intentioned, but they did not get a handle on my illness and I was not able to help to them. One of the things I've learned about lupus is this: patients' coping skills may be compromised. Fatigue is a hallmark of the disease, and so, often, is muddled thinking (it's called "lupus fog").
A few years ago I was referred to a rheumatologist who tweaked my treatment plan. Because I do not have a vicious form of lupus, that's all it took for me to become productive once again. Very quickly, with effective care, my intellectual focus returned and I began to work.
First I tried to read; I grappled with strange words, new concepts. Then I started to write. I had been a teacher, so summarizing information in digestible nuggets was already part of my skill set.
My first book about lupus was published a year and a half ago. Since then I have maintained a web site devoted to getting the word out about lupus research, treatments, etc. I have put together this small pamphlet. I blog occasionally on different lupus sites, and I am currently planning another small book, which will add to the information in this pamphlet.
Good medical care has enabled me to pursue my ambitions. I know, from my own experience, that the level of care I receive is not universally available. What I hope to achieve with my various writings is this: all patients should know enough about their disease to ask important questions and to understand the answers to these questions. All patients should have the confidence to seek a second and third opinion, if the first is not working for them. I hope my writing plays a small part in helping patients to achieve this goal.
Introduction
This pamphlet is intended to give an overview of information which might be useful to patients recently diagnosed with lupus--or to those who are currently being evaluated for the disease. Lupus treatment, diagnosis and care are discussed at greater length in my book, A Lupus Handbook: These Are the Faces of Lupus, which was featured in Lupus Now, the Lupus Foundation's official magazine.
As I prepared this small pamphlet (Lupus Digest Basic Facts), I mined some of the material from my larger book and some material from my website (thesearethefacesoflupus.com). Some material is unique to the pamphlet.
A while back, when I first decided to write about lupus, I knew there was a great deal of information already in the marketplace. However, I had been ill and I had been helped. I also had a fondness for technical material. I combined these factors into what I believed to be a unique, useful package.
While usually I work pretty consistently at writing, sometimes I have to take a break because lupus is still a factor in my life. I begrudge every wasted moment. I am aware of time lost and of a finite future. I relish my good health, when it is present. What I hope to achieve with this pamphlet, and all my writing about lupus, is to give others a chance to enjoy the same gift of productivity I currently enjoy and to realize whatever private ambitions they may harbor.
Reading a book, mine or any other, is just the first step on the journey toward better health. New information comes out all the time and is available, for free, on the Internet. Maybe this information will not change your current treatment plan, but having it will likely give you confidence and increase your sense of control.
I try to keep up on my website with the latest information, but obviously I am not able to provide a comprehensive overview of lupus. Nobody is--not even the experts. The disease is complex--researchers describe it as being heterogeneous, which means it takes many forms. Its causes also seem to be rooted in many factors. What I can do, with you, the reader, is eavesdrop on conversations researchers are having about this confounding disease. We can strive, with the knowledge we gain, to become actors, and not passive recipients, in the delivery of medical care.
Chapter 1
Lupus-What Is It?
Autoimmune disease: auto, from the Greek, means self (as in automobile: a self-moving vehicle). An autoimmune reaction, in which the body essentially makes itself sick, can be as simple as a rash induced by contact with poison ivy, or a full-blown case of systemic lupus.
The analogy to a car is helpful here: a car (automobile) operates automatically only in the sense that someone turns it on, turns it off and directs its path. However, from time to time news reports appear that tell us of cars out of control--cars that somehow accelerate on their own or fail to respond to instructions to stop. These automobiles, instead of being dutiful and useful servants, become dangerous agents. This is sort of what happens in an autoimmune disease.
The immune system exists to protect the body. A complex set of interactions go into play when the body is under assault. The interactions include surrounding and consuming an alien entity (such as bacteria). The trigger--or ignition, if comparing it to a car--for this chain of events to happen is an external threat. In a healthy individual, when the threat is removed the immune response--the chain reaction--stops.
However, when autoimmune disease takes hold, the immune system goes haywire, just like a car out of control. Immune agents start attacking tissue that isn't foreign. In lupus, the attacking immune cells can go after any part of the body--the lungs, heart, blood, brain, etc.
The reason why this happens is only vaguely understood. Some of the triggers that set off the autoimmune attack have been identified--Espstein-Barr and UV exposure, for example. But on the whole, the autoimmune syndrome that is at the heart of lupus remains a mystery. Once this mystery is solved, then a cure, or even a way of preventing the disease, may be found.
There are different types of lupus: SLE (systemic lupus erythematosus), SCLE (subacute cutaneous lupus erythematosus), DLE (discoid lupus erythematosus), and NLE (neonatal lupus). These different expressions of lupus can overlap. Some medications are more effective for one type of lupus than for another. As in every situation, patients should find out as much about their diagnosis as possible and should understand the medical regimen recommended to deal with their illness.
It is possible for lupus to be brought on by sensitivity to a drug; this is called drug-induced lupus and is usually reversible when use of the drug is discontinued.
Right now, medicine can offer people who have lupus only one thing: possible control of their symptoms. In most cases this is achievable, though sometimes at great cost. Lupus can be very resistant to treatment. Since the disease is still a mystery and since the remedies for the illness are diverse and variably effective, it makes sense that someone with lupus should seek out the smartest, most skilled physicians available. Although a rheumatologist will likely oversee the care of a patient with systemic lupus (and a dermatologist likewise will treat a patient with forms of lupus that affects only the skin), it may be that a person with systemic disease will be referred to several specialists over the course of their illness. All of these doctors should have one thing in common: they need to be the smartest, most respected practitioners in their fields. Lupus can be a formidable enemy; the patient should have the best medical care possible to meet its challenge.
Chapter 2
Lupus Symptoms
Many diseases are difficult to diagnose. One of these is systemic lupus. There is no protocol for diagnosing lupus that is entirely objective--although objective tests do help doctors arrive at a diagnosis. To a great extent, the diagnosis and treatment of lupus depends on the treating physician's subjective impression. Thus, a patient may consult three doctors and from each receive a different interpretation of symptoms and medical tests. Until the underlying cause of lupus is better understood, this imprecise and sometimes harmful diagnostic process will continue.
A survey of journal articles reveals a complex variety of symptoms that confound interpretation, even for those physicians most expert in treating lupus.(See an article at the Lupus Foundation of America's site: Diagnosing Lupus).
Rheumatologists (lupus is considered to be a rheumatological disease) generally depend upon a list of eleven diagnostic criteria in order to determine if someone has lupus. However these criteria were developed for research purposes and not clinical practice. But since doctors have few other measures to use, they employ these criteria to diagnose the disease. The criteria are periodically reviewed and have changed over the years. For example, Raynaud's Disease used to be on the list but is no longer included. If four of the elements are present, a doctor may conclude that a patient has lupus. The list includes the following elements:
Malar rash, which is generally raised and spreads across the nose and cheeks almost in the shape of a butterfly.
Anti-nuclear antibodies, or a positive ANA.
A discoid rash, which occurs most commonly on the scalp and face and which is scaly, raised and appears in patches.
Photosensitivity, which results in a rash after exposure to sunlight
Two or more joints that are swollen and painful.
Mouth sores, usually painless
Serositis--an inflammation of the heart, lung lining or abdomen.
Kidney disease
Psychosis, seizure or other indication of a neurological disorder.
Blood Disorders, such as reduced numbers of platelets, white blood cells or red blood cells.
Abnormal Immune System Function, determined by laboratory tests. This includes double-stranded DNA (highly indicative of lupus), anti-Smith (found in virtually no other disease) and anti-phospholipid.
Some of the eleven diagnostic criteria are considered more persuasive in arriving at a lupus diagnosis than others. For example, anti-smith antibodies (from a blood test) are just about determinative in diagnosing the disease. And double-stranded DNA (also from a blood test) is also considered highly suggestive of lupus.
Chapter 3
Possible Causes
If the cause of lupus were known, there likely would be a cure. Every year researchers come closer to understanding the physiological mechanism of lupus; every year they get closer to understanding why this mechanism is triggered. It is certain that genetics plays a role in development of the disease. And it is certain that environment also plays a role. When the interaction between environment and genetics is clearly laid out, then science will likely be able to offer the one thing every lupus patient hopes for: a cure.
Right now, although there is not one known cause for lupus, there are environmental factors that have been implicated in the development of the disease. Several of these are discussed below.
Photosensitivity: Negotiating a planet in which light is the source of life is strategically challenging for someone to whom UV radiation is noxious. However, with information and planning, life away from light can be managed. Photosensitivity is a condition in which an individual has an adverse reaction when exposed to UV radiation. Artificial sources of light (without UV), indirect illumination and UV screens facilitate a life in the shade.
It is likely that I have been UV sensitive all of my life, although my “allergy” has only been quantified in the last twenty years or so. When I was a child my siblings used to taunt me with a chant: “fun in the sun”. Apparently I did not enjoy activities that were carried on for long periods in bright sun shine. As I grew older, I sought shade as a natural place of comfort. Pictures of me with my children as infants reveal a woman squinting against the sun under a wide-brimmed hat.
The first time a physician suggested I was reacting to UV was when a dermatologist noted that a rash that had broken out followed like a chart the areas of my skin that had been exposed to the sun. Another dermatologist told me to wear sun screen year-round because of a pattern of discoloration that had become evident on exposed areas.
There came a time when I was diagnosed with systemic lupus. It was not surprising that photosensitivity was one of the manifestations of my disease. A lot of things became clear to me--why I would break into a sweat when I was in stores with bright overhead lighting. Not only would I sweat but my cheeks would redden and my discomfort would grow so that I had to flee from the store, sometimes leaving my as-yet-unpurchased items behind.
Of course, as people who are photosensitive know, reacting to UV is not simply a matter of feeling uncomfortable. It is not even a matter of getting a little rash. UV exposure makes us sick. And that's the part where public perception comes in. That's the part other people don't get.
When I say, “I have to get away from these fluorescent lights,” I don't mean I might get a little headache. I mean a disease process will be activated.
When I wrote my book (A Lupus Handbook: These Are the Faces of Lupus) about lupus, one of the things that prompted me to write the book was  the story of Hannelore Kohl, the wife of the (ex) German Chancellor, Helmut Kohl. Hannelore Kohl committed suicide. Every news item I read described her death in the same way: she had an allergy to “bright lights” and had been living in seclusion because of this allergy. She was depressed by her situation and so she took her own life.
Photosensitivity is included in the ACR’s list of diagnostic criteria for lupus. Estimates for the number of lupus patients who experience photosensitivity vary. Dr. Lenny Tuffanelli writes in the Maryland Lupus Foundation Newsletter, “Thirty to forty percent of lupus patients are truly photosensitive”, though he cautions also that all people with lupus “may be harmed by excessive exposure”.
Most of the estimates about the occurrence of photosensitivity are taken from the ACR, and implicit in these estimates are issues of reporting and interpretation. As with the larger problem of lupus diagnosis and treatment, the physician is dependent upon a patient’s impression, and the physician adds to the mix a clinical filter. It should not surprise anyone that the results of such a process are unreliable. For example, how precise is an estimate which ranges from thirty percent (Dr. Tuffanelli) to seventy-five percent (upper range of ACR estimate)? How can a doctor, or patient make a decision based on such vague numbers?
Dr. Victoria Werth, of the University of Pennsylvania Medical School, explains the process that causes a photosensitive reaction. Dr. Werth and her research team at Penn Medical School have “identified a variant of the human gene for tumor necrosis factor-alpha as a cause of photosensitivity in lupus.” Dr. Werth believes that identifying the gene variant that causes photosensitivity can help doctors to identify people who are likely to get lupus and can also contribute to an understanding of why the disease develops.
Dr. Werth discovered that in a those lupus patients who have one or two copies of the variant gene, exposure to sunlight stimulates the gene and subsequently the skin cells to undergo apoptosis (cell death). The occurrence of cell death is then a trigger for immune system activation.
The photosensitivity of lupus patients (and of others who have diseases the induce photosensitivity) has a pervasive effect on lifestyle. Pronouncements about the manageability of the condition with the use of sunscreens are ill advised. For one thing, sunscreens can be treacherous. My last experiment with one that I bought in Walgreens caused my face to swell so that I looked as though I was suffering from dropsy. Not only did my face swell, but my whole system was put into overdrive and I was ill for about a week.
One well-intentioned site recommends patch testing a new product. Patch test with extreme caution and maybe even medical supervision. I patch tested my new sunscreen twice, once on the inside of my arm and once on the outside. I waited at least 12 hours between tests and another twenty four before putting the lotion (liberally) on my face. Approximately eighteen hours after the last application I started to scratch. First the chin, then the arm. One side of the arm never reacted and the second only mildly.
Not only can sunscreens be harmful, but they are only partially effective. Sunscreen, long sleeves and a hat--all used together are helpful. But this getup doesn’t mean it's safe to wander around in full sun on a summer afternoon bathed in UV radiation.
I generally do not expect people to make exceptions for me or put themselves out, whether because of lupus or any other problem I have. However, I think that so many people have an issue with UV radiation (not just those who have lupus) that simple structural modifications can be routinized. Inexpensive filters can be placed over fluorescent lights, for example. Certainly in a medical setting this should be mandatory.
Epstein-Barr and Lupus: As pieces to the lupus puzzle are discovered and rearranged, the role of a viral antagonist comes to the fore. Among the several specific viruses which have been associated with systemic lupus, one of the most consistent actors has been Epstein-Barr. While as much as 95% of the population (in the U.S., anyway) eventually will be affected by this virus, in most cases infection is an event without noticeable consequence. For a small number of people, however, Epstein-Barr can be a serious illness. Mononucleosis afflicts about 2% of those who contract Epstein-Barr,(see a discussion of this in the New York Times: Students Still Getting Mono After all these Years). And systemic lupus, according to the NIH, can actually be induced by infection with Epstein-Barr (see: Epstein-Barr virus Infection Induces Lupus Autoimmunity, on the NIH website).
To be clear about what this means, let me restate the case for an Epstein-Barr/Lupus link: Epstein-Barr infection has been shown to be one provocation of the immune system which can eventually lead to systemic disease. The process by which this immune system antagonism takes place is spelled out in the NIH article cited above. According to that article, “the first lupus-specific autoantibodies arise from particular antibodies directed against Epstein-Barr virus Nuclear Antigen-1 (EBNA-1) and that infection with Epstein-Barr virus (EBV) is an environmental risk factor for lupus.”
Understanding how someone goes from having normal immunity to having autoimmunity is critical in unraveling the lupus puzzle. The researchers at NIH believe they have insight into the path from normal immunity to autoimmunity, at least when it comes to infection with Epstein-Barr. The researchers posit that the presence of Epstein-Barr stimulates the production of an antibody called “anti-EBNA-1”. Anti-EBNA-1 apparently attaches itself to “lupus-specific autoantigens (Sm or Ro)”. In a person susceptible to lupus, the autoimmune trigger has, in effect, been pulled. After the Anti-EBNA-1 antibodies and the lupus-specific-autoantigens meet up, a cascade of immune system events occurs which leads to the development of lupus.
Of course, the NIH article which spells out the Epstein-Barr/lupus association is laden with terms like “suggest” and “seems”. Much like Agatha Christy's dogged detective, Hercule Poirot, NIH scientists are on a trail. The clues they have assembled are persuasive, persuasive enough for them to conclude, “In aggregate, these results are consistent with an immune response against Epstein-Barr virus being important in at least some patients for the initiation of lupus autoimmunity”. That's a pretty compelling statement, considering the usual semantic reserve of the research-oriented scientific community.
What does all of this mean to someone who has lupus? As in every instance where an association between lupus and an antagonist is established, we may not know if this connection explains our individual experience. What we do know, though, is that in the future others, perhaps members of our own families who share our genetic make-up, may benefit from the understanding that comes from sorting the various clues to the lupus puzzle.
Lupus and Pesticides: While there will always be controversy, particularly between health researchers and industry advocates, about the kinds of environmental exposure that are harmful, all I can do as a non-scientist is report what the research indicates.
There are idiosyncratic responses to every kind of exposure; this means that you never know how something will affect you, individually. The best each of us can do is make informed decisions about the risks and benefits of any substance we allow into our personal space.
The substance under consideration in this essay is HCH, which is used commercially as a pesticide on produce and by the retail consumer as a lice remedy. Lindane, as the louse formulation is known, has certain product advisories printed on the label. Although these warnings can be daunting in themselves (potential for seizures and other neurological events, for example) what is not mentioned is the possible exacerbation of SLE. According to an NIH document (Alterations in T-lymphocyte sub-set profiles and cytokine secretion by PBMC of systemic lupus erythematosus patients upon in vitro exposure to organochlorine pesticides): “Chronic exposure to organochlorine pesticides (OCP) has been suspected of causing immunoregulatory abnormalities that eventually lead to development and progression of systemic lupus erythematosus (SLE), but the role of these non-genetic stimuli has remained poorly understood.”
The “organochlorine pesticides” referenced in the NIH article include HCH and DDT. Important for us to note in the NIH report are the words “chronic, suspected and poorly understood”. Also essential for us to take note of is the phrase “non-genetic stimuli”. That means this effect, if legitimate, isn't a legacy of our ancestors or our gene pool. It's something that definitely happened to us after we were born. That's good news. Because that is something over which we may have some control.
Researchers have always suspected that lupus was linked to a genetic predisposition yet was not determined by that predisposition. Studies of identical twins pretty much prove this. While there is a high probability that if one twin has lupus the other will also, many cases exist where there is an affected twin and one that is not affected.
So scientists, and we, can look to the environment to understand why we may have lupus. What happened to bring on the lupus? If there are antagonists in the environment, then maybe we can try to remove these. If HCH is a lupus antagonist, then that is something we should know and when a doctor recommends that we use it to rid ourselves of a lice infestation, maybe we should think long and hard about this and look to another remedy.
Insecticides have always been a quick and convenient way of dealing with insect infestations. While these products may still have their place, perhaps we should be certain that they are a last and unavoidable line of defense.
I know I will. I cannot change my past. I grew up in an agricultural community where crops where regularly “dusted”. I had children whom I treated with preparations to rid them of lice. I used these preparations myself as a prophylactic against lice during these episodes. My dogs had fleas; I treated the house and the animals with insecticides.
I don't know if any of this contributed to my experience with lupus. I do know that in the future I will spend more time and energy looking for alternatives to what in the past seemed like an efficient solution to a pesky problem.
Periodontitis and Lupus: I've been looking at the possible association between SLE and periodontitis because of a short article I read on the University of Birmingham (U.K.) site. On this site Dr. Iain L.C. Chapple refers to his work investigating  the relationships between periodontal inflammation and systemic inflammatory diseases.
There are, it seems, two major types of gum inflammation: one is caused by plaque formation and irritation. This is called gingivitis and is the kind of gum disease most people experience; gingivitis is usually resolved by prophylaxis in the dentist's office. In a small percentage of cases, unresolved gingivitis leads to periodontitis, which is a more serious condition. Periodontitis is a chronic, inflammatory, autoimmune response to pathogens. With gingivitis, inflammation is localized; tooth structure and underlying bone are not involved as they are with periodontitis.
The disease process in periodontitis is similar to that in other autoimmune diseases and the condition is just as recalcitrant to treatment. As with systemic autoimmune diseases, the condition can wax and wane and responds more or less to to medical intervention on a case by case basis.
While I could find only a very weak, if any, association between periodontitis and systemic lupus, there was a strong correlation between this kind of gum disease and other systemic inflammations. Autoimmune periodontitis has been implicated in the development of rheumatoid arthritis. It has also been associated with atherosclerosis and diabetes.
Periodontitis has also been associated with end stage renal disease.
Although the evidence I uncovered for a link between SLE and periodontitis was not strong, research has established that a correlation does exist between autoimmune gum disease and acute cutaneous lupus erythematosus.
It's tempting, when finding associations between conditions, to suggest that one causes the other. But many of the articles I've cited in this essay are careful to draw a distinction between cause and correlation. In the National Institutes of Health piece on end-stage renal disease, for example, the authors point out that it is not yet determined whether treatment of periodontitis will affect the course of renal disease. On the other hand, in the instance of the diabetes/periodontitis correlation, the author of the NIH article suggests that causation goes both ways: having type 2 diabetes predisposes someone toward development of periodontitis and chronic periodontitis can instigate kidney inflammation.
How does this help us, besides adding to our understanding of our bodies? Established protocols for treating autoimmune diseases depend heavily on immune suppression. This brings with it a host of problems, as we all know. A significant possibility exists, we learn now, that specific pathogens (infectious agents) may be implicated in the development of lupus. Therefore, instead of suppressing the entire immune system--which is kind of like spraying shotgun pellets at a scurrying mouse--we might be able to target specific antagonists.
Wouldn't that be a wonderful revolution in lupus treatment?
Staph and Lupus: Researchers at the Mayo Clinic have discovered that common staph infections can precipitate a lupus-like disease in mice. According to Dr. Vaidehi Chowdhary, this discovery opens the possibility for the development of new therapies for lupus and even, conceivably, for the prevention of the disease. He explains that if human studies yield the same results as mouse studies, then individuals who are genetically susceptible to lupus may avoid developing the disease altogether if they eliminate staph from their environment. While this is only conjecture at this point in the research, Dr. Chowdhary believes the staph/lupus association is an exciting breakthrough. The study was published in the August 2012 issue of The Journal of Immunology.
Chapter 4
Lupus Treatment
Lupus is a disease with a wide range of symptoms. Although the disease can behave in predictable patterns, it also can be so idiosyncratic that doctors are surprised by some of its manifestations. Since there is no cure for lupus, the emphasis is on controlling the disease, on suppressing the symptoms and hopefully forcing the disease into remission.
Doctors have a wide range of medicines to achieve these goals. The first line of defense begins with the patient. Strive to reduce stress, increase rest, improve nutrition and stay away from lupus antagonists, such as UV radiation. (I recognize that each of these life style modifications may be difficult, if not impossible for many people).
After the patient has done everything possible to defend against the disease, doctors turn to the prescription pad. For mild lupus, which consists of arthralgia and arthritis, the doctor may prescribe first a non-steroidal anti-inflammatory.
NSAIDs--a non-steroidal anti-inflammatory, such as Ibuprofen or aspirin. These are the most benign medications available--though they are not benign. There are a host of side effects associated with these, including heart problems, blood disorders, liver issues and gastrointestinal problems.
If the NSAIDs are deemed insufficient to control the symptoms of lupus, there is another class of drugs the physician can turn to. These generally suppress immune activity. Five of the most commonly prescribed immunosuppressive drugs are described below. In each case, I describe the effect the medicine is supposed to have on the course of lupus and I list possible adverse consequences of taking the medicine. My description is superficial at best. It is only meant to give the broadest overview of these very powerful compounds. If you are contemplating taking any combination of these, do you homework. Read up on them and have discussions with your doctor.
Prednisone: If you have lupus, you likely will be introduced at some point in the course of your disease to prednisone, or one of its pharmacological cousins. Prednisone is a glucocorticoid, a synthetic form of cortisol, a hormone that occurs naturally in the body.
In 1950 two physicians from the Mayo Clinic received the Nobel Prize for synthesizing cortisone, a prednisone precursor, in the laboratory. This drug has improved the lives of many people who have lupus and people suffering from other conditions.
Today, glucocorticoids remain the cornerstone of lupus treatment, though a host of other drugs have been found effective in controlling the disease. While steroids can often control symptoms of lupus, they are notorious for the side effects that may accompany their use. Researchers are still examining, after all these years, the unintended but often unavoidable consequences of therapeutic steroid use.
There is always a certain amount of cortisol (the naturally occurring steroid) circulating in the bloodstream; in an emergency, the adrenal cortex is prompted to release more. The increased cortisol marshals the body's resources to handle a crisis. Among other things, the immune response is suppressed, heart rate and blood pressure are increased. We all have experienced this; it's part of our fight or flight syndrome and it is a stress reaction over which we have little conscious control.
In mimicking the behavior of cortisol in the body, prednisone likewise reduces the immune response (which in an autoimmune disease like lupus is out of whack) and thus reduces inflammation. Unfortunately, prednisone is not a targeted medication. It doesn't just go after inflammation; it affects a multitude of organs. Some of the potential side effects of steroid therapy are: cataracts; diabetes; pancreatitis; acne; bone loss (osteoporosis); bone necrosis; hair loss; hair growth; adrenal insufficiency; high blood pressure; weight gain (especially trunk); psychosis; insomnia; blood clots; immune deficiency; glaucoma; ulcer; long-term migraines; mouth sores; joint pain; facial swelling; gastric bleeding; atherosclerosis.
Because of the diffuse and profound effect that steroid therapy has on the body, discontinuation is accomplished gradually, through a “weaning” process. The adrenal cortex has to, in effect, be trained once again to start producing cortisol. Failure to gradually wean off steroids can be very dangerous.
Recent research suggests that doses of prednisone below 90 mgs a month are not likely to result in organ damage; however, this finding is not certain.
Some of the conditions for which prednisone has been found to be effective are: Crohn's disease, lupus, rheumatoid arthritis, Non-Hodgkin's lymphoma, Hodgkin's lymphoma and sarcoidosis. While many autoimmune diseases respond to prednisone, it has to be used with great caution in the treatment of scleroderma because of the risk of acute renal crisis.
One of the ways prednisone is effective in tamping down the immune response is by suppressing cell proliferation. A challenging aspect of prednisone therapy is that it has to potential to precipitate some of the very disorders it is used to treat. For example, while mania may be a manifestation of lupus and may successfully be addressed by administration of prednisone, there is also a form of mania which can be caused by taking prednisone. Also, prednisone may be prescribed to treat cluster headaches or migraines, and yet long-term use of prednisone has been implicated in causing chronic migraine.
It has been noted that pregnant women who had been on steroid therapy during their first trimester have a greater chance of bearing offspring with cleft palate.
Often, steroids are used in conjunction with other immunosuppressive agents; sometimes this is done to increase the effectiveness of therapy. Alternately, dual therapy is often introduced so that a patient may be “spared” steroid exposure.
As with every medicine, prednisone therapy can have profound and irreversible effects. However, for the treatment of systemic lupus, or other debilitating diseases, prednisone can be a life-saver. Understanding its benefits and risks is essential for a patient who has to cooperate in treatment decisions.
Plaquenil: One of the first medications a lupus patient may be offered is Plaquenil. While every drug has side effects, Plaquenil is generally considered to be “safer” than many other medicines prescribed to treat lupus.
Researchers are finding out more about this drug all the time; the current state of science indicates that Plaquenil is effective against lupus, and some other inflammatory diseases, because it creates an inhospitable environment for some actors in the immune process.  In this way Plaquenil inhibits the immune response and thus also inhibits inflammation.
Though Plaquenil is considered to be relatively “safe”, there are several populations in whom great care should be taken before medicine is prescribed. If you are over the age of 60, the chance for retinal damage increases. If you have psoriasis or porphyria, taking this drug may exacerbate your condition. Anyone with reduced liver or kidney function should also consider carefully if the risks of the drugs outweigh its benefits. Plaquenil can be extremely dangerous for children, especially children under the age of 6. People who have a metabolic disorder called G6PD run the risk of developing severe anemia on Plaquenil therapy.
The most commonly addressed side effect Plaquenil therapy is retinal damage. Every responsible opinion I have read prescribes a visual screening before therapy begins and then a re-check several weeks later. While it is rare for eye damage to occur, especially if therapy lasts less than five years, it is important to keep in mind that once damage occurs, it is most likely irreversible. The changes in the retina may not be noticed subjectively but can be detected by sophisticated testing in the ophthalmologist's office. Many doctors recommend a yearly check-up; some recommend six months. If I were on Plaquenil I would go for six months because I'd like to detect damage before it goes very far.
With all of the warnings listed above (this is just a partial list of possible side effects) it would seem that Plaquenil is a dreadful drug. It really isn't for most people. Lupus is a dreadful disease and sometimes you have to take out a big gun to control it. Plaquenil is actually one of the smaller guns in the arsenal against lupus.
Originally used as a treatment for malaria, Plaquenil is useful not only to treat SLE (though not severe SLE), but also other inflammatory diseases. It is supposed to be particularly effective at treating discoid lupus.
All of the sources I consulted implicate higher-dose and longer-term Plaquenil treatment in increased risk of eye damage. So if you are on Plaquenil for many years, certainly as many as 8, your doctor might begin a conversation with you about switching to an alternate treatment.
Methotrexate: Methotrexate is an immunosuppressive drug—which means it interferes with the production of some kinds of immune cells. Back in the 1950's doctors noticed that patients who had certain types of cancer did better if they were deficient in folic acid. This observation led to the development of methotrexate, which interferes with the action of folic acid. Treatment with methotrexate became common for selected cancers because the medicine was found to have less side effects than some other preparations.
Eventually, methotrexate was discovered to be useful in suppressing immune activity in autoimmune diseases, especially rheumatoid arthritis, but also lupus and Crohn's disease, among others. Doses necessary to achieve a therapeutic effect in lupus are much lower than those administered in the treatment of cancer. Consequently, side effects may not be as severe.
However, methotrexate is a powerful drug and can have very serious side effects. It is absolutely prohibited if there is a chance of pregnancy. Even after discontinuing use of the drug, pregnancy must be delayed until all of the medicine has cleared from the system. There is the possibility of liver and lung damage while on this drug. Also, nervous system complications have been noticed in some cases.
Drug interactions include penicillins, probenecid, phenobarbitol, carbamazepine, Bactrim and NSAIDs  (although NSAIDs may sometimes be prescribed in combination with methotrexate). It is recommended that while on Methotrexate the patient abstain from drinking because of possible damage to the liver. It is essential that the patient be monitored (blood tests) regularly. Any sign of shortness of breath or chest discomfort should be reported immediately to the doctor.
Patients taking methotrexate often are prescribed a folic acid supplement to avoid deficiency.
Though Methotrexate is widely used for the treatment of lupus, it is generally not the medicine of choice for those who have lupus nephritis or organ involvement. According to the Mayo Clinic, methotrexate should be used with caution in lupus patients with these symptoms.
While methotrexate is a powerful drug and can have potentially serious side effects, the fact remains that it has been very useful in the treatment of a variety of diseases. As with every medicine, risk has to weighed against benefit. For many patients, the benefits are greater than the risk.
Mycophenolate: It is estimated that between 50-60% of lupus patients will experience nephritis at some point in the course of their illness. The numbers vary by ethnic group and gender. For example: men, Hispanics and people of Afro-Caribbean origin tend to get nephritis in greater numbers and are inclined to have a more severe course of illness than members of some other groups.
Outcomes for people with lupus nephritis have improved dramatically over the years. This is partly due, it is believed, to more accurate diagnosis and also to more effective treatment.
In 2012, The American College of Rheumatology issued new guidelines for treatment of lupus nephritis. This is the first update issued by the ARC since 1999; the new guidelines reflect the results of many clinical trials. The ACR describes five classes of lupus nephritis, with I being the mildest and V being the most severe. Depending on the class into which a patient's disease falls, treatment recommendations vary. For class III, IV and V an immunosuppressive agent, in conjunction with prednisone, is recommended.
Clinical trials demonstrate that the use of mycophenolate produces a higher rate of improvement, for some patients, than some other therapies have produced.  Of course, doctors must assess the way in which each patient responds to therapy. Treatment protocols need to be to be adjusted accordingly.
Because mycophenolate is a powerful immunosuppressive agent, the risk of developing an infection or for an infection to worsen is increased. So it is important for patients to be alert to any change in health status, whether that change be a blister, sore throat or fever. The doctor should be consulted as soon as an issue is detected.
There are many potential drug interactions with mycophenolate. The doctor should be informed about any preparation the patient might be taking, including herbal supplements and vitamins. Anyone who is allergic to aspartame should tell the doctor about this because it seems aspartame is included in the mycophenolate suspension.
Mycophenolate tamps down the immune system by suppressing cell proliferation, especially proliferation of B cells, which are prime actors in lupus nephritis.
Mycophenolate has been shown to cause birth defects, especially in the first trimester. Any woman of child-bearing age must use two methods of birth control while taking this medicine and she must continue with the birth control regimen for weeks after stopping the medicine—a doctor will advise as to how long this should be.
Although mycophenolatte is a powerful drug which has the potential to cause serious side effects, the drug can be a life-saver. Nephritis should never be underestimated. No patient should be casual about this manifestation of lupus. Early and aggressive treatment is often the to key to a successful outcome. With lupus, it's always recommended that the patient consult closely with the doctor about any changes in health status. With lupus nephritis, especially when on immune suppressive therapy, the principle under which a patient needs to operate is: keep your doctor informed.
Resources the patient might read:
ACR Guidelines for Screening, Treatment and Management of Lupus Nephritis:
http://www.rheumatology.org/practice/clinical/guidelines/Lupus_Nephritis_Guidelines_Manuscript.pdf
Nature: Should Mycophenelate Mofetil Replace Cyclophosphamide?:
http://www.nature.com/ki/journal/vaop/ncurrent/full/ki2012203a.html
Azathioprine: In my brief article on Plaquenil, I mention that there are bigger guns than this medicine in the rheumatologist's lupus arsenal. One of the bigger guns is azathioprine. Azathioprine is an immunosuppressant which was designed in 1957 for use in chemotherapy; however, the drug was discovered to be useful in the treatment of some autoimmune diseases, including lupus.
Although today azathioprine is widely accepted as a lupus treatment, the FDA has approved the drug for only two uses: kidney transplants and rhuematoid arthritis. All other uses are considered “off-label”.
Nonetheless, azathioprine can be a powerful ally in the battle against lupus. The drug acts to inhibit cell reproduction, especially reproduction of T and B cells, two lymphocytes implicated in inflammation. Often, azathioprine is given in addition to a corticosteroid. When azathioprine starts to take effect (which may not be for months) steroid use may be reduced or even eliminated.
People taking allopuranol, niacin, warfarin and certain muscle relaxants should be aware that potentially dangerous drug interactions have been observed between when these medicines are taken in conjunction with and azathioprine. There is also some controversy about whether an statistical increase in fetal abnormalities is due to azathioprine use or to the underlying conditions for which azathioprine was prescribed. According to the Mayo Clinic: “Studies in pregnant women have demonstrated a risk to the fetus”. The Mayo site goes on to say that even though this risk exists, in some cases there has to be balanced the risk to the fetus against the risk to the mother's life.
The chances of developing skin cancer rises with azathioprine use, as does the likelihood of developing a rare type of lymphoma. There is also the potential to experience bone marrow suppression and leukopenia. These are just some, not all, of the potential side effects of azathioprine therapy. That said, this drug can be very useful and effective; it is taken successfully by many people. I came across the abstract of an article which describes the success rates for patients who were treated with azathioprine (see Arthritis and Rheumatism, below). People who had begun the drug course with a poor prognosis showed a dramatic increase in survival rates and those with a good prognosis showed a dramatic decrease in hospitalizations.
Although reading about the side effects of a medicine may be upsetting, that shouldn't dissuade anyone from accepting proper, sometimes aggressive treatment for their disease. As I describe in my book, A Lupus Handbook: These Are the Faces of Lupus, appropriate medical interventions, including powerful drugs, may be all that stands between a lupus patient and good health.
Some informative sites:
Mayo Clinic:
http://www.mayoclinic.com/health/drug-information/DR601561
Data Sheet, New Zealand Gov't:
http://www.medsafe.govt.nz/profs/datasheet/i/imuprinetab.pdf
Arthritis and Rheumatism:
http://onlinelibrary.wiley.com/doi/10.1002/art.1780180106/abstra
A Word on Plaquenil and Quinoric: I've read many posts on Lupus UK which raise the question of whether Quinoric is as effective as Plaquenil in treating lupus. Several people on the site suggest that the only difference between the two drugs is in the “filler” added to the active ingredient, hydroxychloroquine. And several people add that their pharmacist, or doctor, has assured them the preparations were “identical”. Which suggests to me a larger issue: are generics and substitute formulations for established drugs truly “identical” and do they behave identically when administered?
In an effort to examine the interchangeability of supposedly identical drugs, I took a close look at Plaquenil and Quinoric. Are these two indeed “identical”? Can one drug be substituted seamlessly for the other? I found a list of constituent ingredients for each of these drugs so I could make a comparison.
Both drugs, I discovered, contain the same active ingredient in equal quantity: 200 mgs of hydroxychloroquine. The differences between the drugs, as some people suspected, is in the “fillers”. So I looked at those “fillers”.
Plaquenil (Manufacturer Sanofi). List derived from drugs.com: Calcium Phosphate, Dibasic, Anhydrous, Hypromelloses, Magnesium Stearate, Polyethylene glycol 400, Polysorbate 80, Starch (Corn),Titanium Dioxide
Quinoric (Manufacturer Bristol Labs). List derived from Bristol website: Maize Starch, Calcium, Hydrogen Phosphate dihydrate, Colloidal anhydrous silica, Polysorbate 80, Purified Tale, Magnesium stearate, Hypromellose, Titanium dioxide, Macrogol 6000
While the two lists of ingredients are similar, they certainly are not the same. But the question remains, do these differences matter? I took a look at one ingredient that appears to be almost identical: PEG.
On the Plaquenil list PEG (Polyethylene glycol) is described as Polyethylene glycol 400. On the Quinoric list PEG comes in a different formulation: Macrogol 6000. In industry, these two forms of PEG have different uses because they interact with environments differently. One, Macrogol 6000, is commonly used as a laxative, among other things. The other, Polyethylene glycol 400, has a lower molecular weight and is more liquid: this chemical is often used in printer ink, as well as in pharmaceuticl preparations. It's impossible for me to say if these two forms of PEG are processed identically in the human body.
There's one “filler” included on the list for Quinoric which is not included on the list for Plaquenil: silica (Colloidal anydrous silica). Silica is abundant in the environment. This compound has been linked to a number of autimmune diseases. Whether inhaled, absorbed through the skin or ingested orally, silica has a well-established association with lupus and rheumatoid arthritis, among other other inflammatory conditions. Silica is almost ubiquitous in the marketplace. It's in soaps, cleaning products, paints and other common household items.
Some sources that discuss silica's role in autoimmunity are:
The NIH website at:
 http://ntp.niehs.nih.gov/NTP/Noms/Support_Docs/Si
Google Books, Environmental Stressors in Health and Disease:
http://books.google.com
I think it's fair to ask, what is silica doing in a preparation that's designed to treat lupus? I can anticipate the response to this question: the substance is inactive and largely inert.
I 'm going to turn to Dr. Phil Lieberman of the American Academy of Asthma, Allergy & Immunology for an answer to this question. Dr. Liebermans is responding to a doctor's query about Macrolog 6000, which is used in a wide variety of pharmaceuticals as “filler” (including Quinoric). Dr. Lieberman states: “--it is best to try and avoid polyethylene glycol as carefully as possible. This is a difficult task given the ubiquitous nature of these substances, but it is clearly the safest strategy to employ.”( see: http://www.aaaai.org/ask-the-expert/anaphylaxis-to-polyethylene-glycol.aspx).
So, even when an ingredient is used only as “filler” and is designed to be “inactive”, it still can have potential consequences for the patient.
Quinoric and Plaquenil are obviously not identical. The differences between them may be small, but in certain individuals those differences can be consequential. Plaquenil and Quinoric are prescribed for a very particular population, a population of people who have heightened immune responses. Allergies, even rare allergies, are common in this population. So even discrete quantities of a unique filler may present a problem in a small percentage of patients taking either of these drugs.
Plaquenil and Quinoric are not identical; they may not be absorbed the same into the bloodstream, or processed in exactly the same way by the digestive system. There may be constituent ingredients that are irritating in one and not the other.
This issue is not unique to Plaquenil and Quinoric; the issue of interchangeability between knock-off drugs and brand name drugs is one that affects every person who has to take medicine. Blanket statements that two medicines are “identical” do not seriously address the issue. This is the truth, and truth is something every patient has a right to expect.
Abbreviated Glossary
Antibodies--An essential part of the immune process. These cells are produced by the immune system to attack foreign substances
Autoantibodies--cells produced by the immune system that attack cells of the producing organism (against self)
Anti nuclear antibodies--antibodies that attack the nucleus of cells
Cerebrovascular disease--disease of the blood vessels in the brain
Collagen--the protein that makes up the majority of connective tissue in the body
Connective Tissue--as the name suggests, connective tissue connects and supports all the organs in the body
Creatine--substance present in the muscles
Differential Diagnosis--method of arriving at a diagnosis used in lupus. This is a process by which doctors evaluate a patient’s complaint (reason for going to the doctor in the first place) and start with the most obvious explanation and through a process of elimination, aided by diagnostic tests, arrives at a conclusion. Differential diagnosis can thus take a very long time and involves interpretation and insight
Discoid Lupus--kind of lupus in which lesions, shaped approximately like discs, appear on the skin. Can also appear in systemic lupus. About ten percent of the people with discoid lupus go on to develop systemic lupus
Drug Induced Lupus--certain drugs precipitate lupus. However, with a discontinuation of the drug, drug-induced lupus goes away
Enzyme--a protein that acts as a catalyst on other substances in the body
Erythema--possible symptom of inflammation; the skin becomes red because underlying blood vessels dilate
Erythematosus--a redness of the skin caused by inflammation of the blood vessels beneath the surface
Glomeruli--small blood vessels in the kidneys which filter waste from the rest of the body
Glomerulonephritis--inflammation of the glomeruli
Immunofluorescence--a laboratory process in which the specimen is stained with a fluorescent dye
Immunosuppressive--something, such as a drug, which suppresses the activity of the immune system
Lymph--occurring throughout the body, it acts as a kind of transportation system. It carries fats, bacteria and other material to the lymph nodes where these impurities are filtered out. It carries lymphocytes, which are white blood cells that are part of the bodies disease fighting mechanism
Lymphoma--cancer that effects the lymph system. Occurs more commonly in lupus patients than in the general population
NSAIDs--non steroidal anti inflammatories, such as aspirin
Neonatal Lupus--when the mother transfers autoantibodies to the fetus in the womb
Nephritis--inflammation of the Kidneys
Neuropsychiatric--psychiatric condition arising from a neurological disturbance
Osteoporosis--thinning and weakening of the bones, often leading to fracture. Can be caused by steroid therapy
Phagocyte--any cell that ingests invading organisms and helps clear cell debris
Photosensitivity--demonstration of reaction to exposure to sun or UV radiation
Pleuritis--serositis of the lungs
Renal--having to do with the kidneys
Renal Failure--when the kidneys no longer filter the blood and toxins build up in the blood stream
Serologic--having to do with the blood
Serositis --accumulation of fluid in the lung, heart or abdomen
Steroid--naturally occurring or synthetic hormone that helps regulate different systems and maintain homeostasis
Subclinical--a disease process that exists but has not yet manifested symptoms. In this case, perhaps a laboratory test will reveal the existence of an abnormality that could become significant in the future or under certain conditions
Titer--level, or measurement of a substance in the blood
White Blood Cells--also called leukocytes, white blood cells are the units in the blood that fight invading organisms (infection)
Laboratory Tests
ALT(SGPT)(alanine aminotransferase)--Tests for injury to the liver. This test, in conjunction with others (ex., bilirubin) can help pinpoint the kind of injury or disease that the elevated levels are indicating. The higher the ALT level, the more acute the problem
Amylase--elevated amylase indicates a problem with the pancreas. The higher the level, the more acute the problem
Antinuclear antibody--blood is tested either by using indirect immunofluorescence or Elisa (Enzyme-linked immunosorbent assay). The Elisa is less accurate but also less expensive. This test detects autoantibodies. If titer if 1:40 or less, this is considered a normal reading.
AST (SGOT)--this test is conducted to discover if liver disease is present. Elevated levels are an indication that the liver has been stressed
Anti dsDNA--several methods are used to detect the presence of anti-- dsDNA. EIR is the more accurate and thus the preferred test. IFA and RIA are also done. If these tests reveal the presence of anti-ds DNA, this is almost diagnostic for lupus. This antibody is found almost exclusively in lupus patients and about 50-70 percent of them have it
Anti SM--this tests looks for the presence of the Anti-Smith antibody. The antibody was found in the blood of a lupus patient named Smith and was named after her. Presence of this antibody is almost diagnostic for lupus
bilirubin--produced by the liver, it is a byproduct of the breakdown of red blood cells. Elevated bilirubin can indicate liver problems
CBC--blood test that determines serum levels of red blood cells, white blood cells platelets and hemoglobin
C-Reactive Protein--a marker for inflammation in the body, not specific. Elevated levels of C Reactive is thought to be associated with an increased risk for heart attack. 
Creatinine (CR)--high levels of creatinine indicate a problem with the kidneys
Eosinophils--a type of white blood cell. Elevated serum values typically are found in allergic reactions and parasitic infections
ESR--erythrocyte sedimentation rate. Elevated levels of ESR is an indication of inflammation.
Fibrinogen--a blood protein necessary for clotting. High or low levels can indicate clotting problems.
Glucose--blood sugar. This test is performed after fasting. Higher than 127 (after a fast) indicates diabetes
Hemoglobin--a blood protein that carries oxygen throughout the body. Low hemoglobin counts indicate anemia, which can be caused by any number of factors. Excess hemoglobin is also a red flag
Hct (hematocrit)--This test is performed to discover what percent of the blood is comprised of red blood cells. Low hematocrit is an indication of anemia
Lipase--high amounts of lipase in the blood is an indication of a problem with the pancreas
Liver Enzymes--the liver secrets a number of enzymes. Elevated levels of these indicate liver stress and warrant further examination
Lymphocytes--white blood cells. In the presence of infection, these numbers are elevated
Macrophage--a phagocyte. Like sentinels, they stand guard at specific sites in the body ready to defend against invading organisms. Detection of an increase in the number of macrophages indicates an elevated immune response
Microphage--a phagocyte, like a macrophage, except more numerous, less long lived, and less specific in activity
Monoocytes--a kind of red blood cell. Elevated monocyte levels can be an indication of an infection and/or of inflammation
Neutrophils--white blood cells. This is the type of white blood cell most commonly found in the body. A variety of conditions lead to high neutrophil counts. Reduced counts indicate increased susceptibility to infection
Occult Blood--this test looks for blood in the stool that is not visible to the naked eye. Any number of conditions can be the cause of blood in the stool. Some, like hemorrhoids, are relatively benign, and others, like intestinal lesions, are not
pH--this test checks the acidity of the blood. The normal range for blood is slightly over seven. If the ph falls under 7.35 the condition is known as acidosis. If the ph falls over 7.45 the condition is known as alkalosis. The body keeps tight control over ph levels. Either acidosis or alkalosis are dangerous
Phagocyte--these are essential to the immune system. They identify and destroy invading microorganisms. Also play a role in development of immunity and  are thought to play a role in autoimmune inflammation
Platelet Count--platelets are essential for clotting. The number of platelets may increase or decrease with infection. Sticky platelets lead to clotting; a deficiency leads to bleeding
Protein--test is ordered to look for a many conditions, usually those of the liver and kidneys
RBC (Red Blood Count)--Usually conducted as part of a CBC (Complete Blood Count) RBC determines the number of red blood cells
Sodium--test carried out to determine the amount of sodium in the blood. A number of medications can disrupt the level of sodium and the results of this test would be one indication
Specific Gravity--part of urinalysis. Tests to see the water to urine concentrations
Uric Acid--excess uric acid in the blood can be a sign of kidney disease
Urobilinogen--A product of the breakdown of bile by the liver. Abnormal levels can indicate liver problems
WBC--white Blood Count. Tests for the number of white blood cells. Normal range is approximately 4,000 to 10,000 per mcL. Too many can indicate infection. Too low can be a sign of immune deficiency