By A. G. Moore

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 the  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 of 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. And 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 in treating SLE (though not severe SLE), but also other inflammatory diseases. The drug 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 alternative treatment.

Several helpful sources I used in order to get information for this article were:

Plaquenil Data Sheet:

I think anybody contemplating taking this medication should read and understand each of these sources. In making this recommendation I am operating on the theory that, though we may not know everything the doctor knows, we always should be vigilant in supervising our own medical care.



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, for most, implies an adverse reaction to UV radiation. Artifical sources of light, indirect illumination and UV screens facilitate a life in the shade.

However, none of these accommodations addresses a major obstacle in negotiating a life away from light.

It is likely that I have been UV sensitive all of my life, although this phenomenon 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 got 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 that a disease process will be activated.

Last year I wrote a book about lupus and one of the things that prompted me to write the book was when I read about Hannelore Kohl, the wife of the (ex) German Chancellore, 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.  Estimates for the number of lupus patients who experience photosensitvity vary. Dr. Lenny Tuffanelli writes in Maryland Lupus Foundation newletter, “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 occurrence 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.  Blithe 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 together are helpful.  But this get up doesn’t mean its 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 florescent lights, for example.  Certainly in a medical setting this should be mandatory.

Neutrophil Extracellular Trap

Neutrophil Extracellular Traps 

By A. G. Moore

This isn’t about basketball or hair management; this is about autoimmunity. It seems that one of the latest cuplrits to be discovered in the search for lupus antagonists is something called the neutrophil extracellular trap(see  Neutophils are the most plentiful disease-fighting cells in the immune system. The role of neutrophils is to attack and kill invading organisms. These highly motivated soldiers in the immune system’s army are willing to commit suicide in order to fulfill their mission. They do this by releasing chemicals between the cells. The chemicals from different neutrophils join together and form a trap, which serves as a kind of net to capture and destroy pathogens. In order to release enough of the deadly material to be effective, the neutrophil has to deplete its own supply and die. It does so willingly.

Unfortunately, this ally of the body, in waging war, sometimes does damage to the very organism it seeks to protect. When the NETs (neutrophil extracelluar traps) are floating around in the bloodstream they can cause inflammation.

The discovery of the role that NETs play in the development of inflammation is very important to understanding the mechanism which precipitates lupus flares. The more researchers understand this process, the closer they get to thwarting the process. Not only may it be possible to design better therapies so that many of the damaging effects of lupus can be avoided; it may also be possible, with an understanding of the role that NETs play in inflammation, to find a cure and maybe even prevent lupus.

Lupus and Vaccines

By A. G. Moore 9/2/2012

People with lupus have a greater susceptibility to infection (see: Lupus Foundation The increased risk for infection arises in many cases from the immunosuppressive drugs that lupus patients take (such as methotrexate, prednisone and Azathioprine). Increased susceptibility can come also from the disease itself, although the degree to which this is true varies according the individual. In a 2005 study, published on the ACR website ( infection rates were compared for hospitalized patients who had rheumatoid arthritis, nephrotic syndrome, and systemic lupus. It turned out that those with systemic lupus had higher infection rates than other patients in the study—even those with SLE were not on immunosuppressive drugs. The ACR article’s conclusion is that the higher infection rate for lupus is not associated with compromised renal function or immunosuppressive drug therapy but to some other aspect of the disease.

So what does a person with lupus do about this increased susceptibility? For one thing, stay away, as much as possible from people who are evidently sick (with an infectious disease). Also, in cold and flu season, keep away from crowds. Simple things, like washing hands and not touching your face, have been proven to be effective infection controls.

Another recommendation, endorsed by the Lupus Foundation, is to keep your vaccinations up to date. However, according to the Foundation, someone with lupus should never receive a live vaccine and should avoid exposure to people who have recently received live vaccine. Also, the possibility exists that if the flu vaccine is given during a lupus flare, then a serious complication may ensue (see:

Personally, I approach flu vaccine season with ambivalence. While I was vaccinated most years without incidence, last year I experienced a pretty serious reaction very soon after being vaccinated. Not only did my arm blow up pretty quickly, but by the next morning I was ill and I stayed ill for about two weeks. Though I dread going through that again, I wonder how much sicker I would have been if I had caught the flu?

As for run of the mill upper respiratory infections, I take a few precautions. First of all, I use avoidance and barriers. I stay away, as much as possible, from people who are evidently sick (with an infectious disease). If I must be around someone who is ill—to nurse, let’s say, a family member— I wear double layer surgical masks (N95 rated) and disposable gloves. I wash the clothing that has been exposed as soon as I am out of the sick room.

If I seem to be coming down with a cold, I pop a Cold-EEZE lozenge into my mouth. The zinc in the lozenge is suppose to reduce the length and severity of the cold—if taken in the first 24 to 48 hours. The most recent study I found that supports the idea that these lozenges are therapeutic, was published in the Journal of Infectious Disease, in 2008 ( see: I usually don’t wait for a cold to develop to start this regimen. I keep Cold-EEZE in the house and as soon as my throat feels scratchy I suck on a lozenge and keep up the regimen till all signs of a cold have disappeared.

The final thing I do for myself is, if I am ill and things don’t seem to be going well, I call my doctor.

I don’t know if any of the measures I take will be helpful to someone else. I state again, as I always do: I am not a doctor. I read a lot and learn from what I read. I hope the information is helpful to others.

Zebras, Not Horses

Zebras, Not Horses

Image from the National Park Service
Public Domain

“When you hear hoof beats, look for horses, not zebras”. This is a principle most doctors learn in medical school. What it means, basically, is that a doctor should look to the most obvious for an answer, not the exotic. This may be a sound diagnostic principle, but for a subset of people who have rare diseases, it is perhaps not the most effective approach to medical care.

I speak, of course, from personal experience. Not only did it take four years for a doctor to decide that I had lupus, but there also was a span of about twenty years when I suffered, without treatment, mysterious episodes of acute abdominal pain. A whole herd of horses was pulled out to explain these episodes. But not until an emergency room physician insisted on taking a CT scan during one episode was a clue to my gut disturbances obtained. And not until a very astute and careful gastroenterologist studied these CT images was the puzzle further unraveled. It finally took my rheumatologist, a  creative and talented physician, to spot the zebra in the herd of horses: he determined that I was experiencing lupus-induced inflammation of the gut.

As idiosyncratic as lupus can be, there is still the conviction among many in the medical community that horses and not zebras predominate. In a Lupus Foundation article, for example, the transcript of a dialogue is presented. The dialogue is between Dr. David Isenberg , a noted gastroenterologist, and lupus patients. Dr. Isenberg explains to the patients the low probability that certain GI conditions will occur as a direct result of lupus. The doctor is asked about ascites (fluid in the abdomen), for example. He asserts that ascites are most likely to be caused by kidney or liver problems.

Of course, my experience refutes Dr. Isenberg’s perspective. Ascites was detected during several of my GI episodes. I did not have liver or kidney disease, although once I did have pancreatitis.

I came across another article which suggests my experience is not unique, though it certainly may not be common. In the medical journal RadioGraphics, which is dedicated to the field of radiology, I found the description of a young woman who had an acute episode of small bowel inflammation, with ascites. The woman also had systemic lupus. Her inflammation was successfully treated with high-dose steroids.

While Dr. Isenberg is an esteemed and accomplished researcher, in his interview he entertains as very remote the scenario suggested in the RadioGraphics article. Dr. Isenberg apparently not only expects to see horses, but he also recommends that everyone else does, too. This is not his “fault”; it is standard medical practice.

I have read that  sometimes ten years can pass between the time lupus is first suspected to the time when it is ultimately diagnosed. Perhaps the reason for this diagnostic lag has something to do with the horse/zebra dynamic. When dealing with an idiosyncratic disease like lupus, which can confuse and confound, maybe doctors should think more often about zebras. Because something is amiss in a protocol which leaves patients, for so long, without appropriate treatment.

I know that in the ordinary course of events, hoof beats will be produced by horses. But sometimes, when the possibility, or strong suspicion, of lupus exists, a doctor might consider the extraordinary. I think a lot of unnecessary suffering and true harm could be avoided if doctors would just allow themselves to look a little bit harder at what seems to be obvious.

A Gene for Autoimmunity?

A Gene for Autoimmunity?

Some years ago I bought stock in a company that investigated the genetic origins of disease. The company went bankrupt and I lost all my money, but there was nothing wrong with the research–just the business plan. Hope of finding a cure–for lupus and other diseases–may rest precisely in understanding the genetic basis of illness.

It was with this idea in mind that a study–A Comprehensive Analysis of Shared Loci between Systemic Lupus Erythematosus (SLE) and Sixteen Autoimmune Diseases Reveals Limited Genetic Overlapwas undertaken. As the title suggests, the study failed to produce evidence that there is a strong genetic link between people SLE and other autoimmune diseases. The authors of the study hypothesize that the correlation may be weak between SLE and many other autoimmune diseases because SLE is so “heterogenous”. That is, there are many ways in which SLE can manifest. Perhaps each manifestation of lupus has a different genetic association.

Interestingly (according to the authors), the strongest genetic association found was between SLE and rheumatoid arthritis. The authors hypothesize that maybe this strong link has something to do with the fact that the two diseases share a major symptom: arthritis. There was another, though weaker, genetic association found between SLE and scleroderma.

The authors also noted that several established genetic links to SLE were not found in other autoimmune diseases. The authors admit that the failure to find these links may be due to a problem with the study’s methodology and that future efforts may be able to detect these associations.

So where do I think this leaves us? Well, there doesn’t seem to be an autoimmune gene, one common cause for a multiplicity of autoimmune diseases. And what the study shows, what it reinforces, is the fact that SLE, even on the genetic level, is a very complicated disease. Systemic lupus is more like several diseases than one and its cure may ultimately involve solving many genetic riddles, not just a single puzzle.

I personally think SLE might be approached the way cancer is increasingly treated: though it falls under the rubric of oncology, cancer responds to a variety of very specific therapies that have been designed for different manifestations of the disease. Something like that may evolve (is already evolving to some extent) for lupus. Though there may not be one gene that causes SLE, there may be different places on the genetic map where a susceptibility for distinct forms of lupus exist. And, just as tailored treatments are developed for different cancers, targeted treatments may need to be developed for different kinds of lupus.

There was a time when certain cancers carried with them very high mortality rates. Some of these, like lymphoma, today have dramatically improved cure rates. The day will surely come when people with lupus will experience the same success. They then will be able to look forward to futures that include the prospect of a complete cure.


NSAIDs: A Cautionary Tale


A Handful of Pills
Image by Ayena

If you have systemic lupus, there’s a pretty good chance you have been prescribed a NSAID (example: aspirin, ibuprofen, Naproxen, etc.) over the course of your illness. Given that likelihood, I have dedicated some time to seeking an understanding of this broad class of drugs. The following essay is one of several I hope to post on the subject.

In writing this post, I have tried to be very careful in my use of language; I draw no conclusions. The available information about NSAIDs indicates that caution should be exercised in their use. Some of the reasons for the caution are explained below. Since research on these drugs continues, the recommendations of the American Heart Association and the FDA for their use reflect only what is currently known. Recommendations of both organizations will likely change over time.

The American Heart Association has issued repeated advisories about the use of NSAIDs; while the Association allows that NSAIDS may still be prescribed under certain circumstances in clinical practice, caution is required. The Association recommends that NSAID use be tempered by an awareness of associated risks. These are risks about which the patient should be informed if NSAID therapy is decided upon. As the Heart Association explains in its report, An Update for clinicians: A Scientific Statement From the American Heart Association (Feb, 2007), the issues surrounding NSAIDS “highlight” the obligation of patients and doctors to balance “the risks and benefits of medications for pain relief”.

So, what are these risks? This is a question that still has not been completely answered. Part of the answer is certainly that there is evidence of increased heart attack, heart failure, stroke and vascular events. The most recent advisory I found from the Heart Association (Sept, 2012) is entitled, “Taking painkillers increases death risk, second heart attack in survivors“. The article  explains that the painkillers in question are NSAIDs. Anne-Marie Schjerning Olsen, the physician who led the relevant study, states: “It is important to get the message out to clinicians taking care of patients with cardiovascular disease that NSAIDs are harmful, even several years after a heart attack”.

According to the Heart Association’s 2007 study (cited above), not all painkillers appear to carry an equal risk of potentially lethal side effects. The Association cautions that results are suggestive and more information needs to be collected about the relative risks of each drug. With this cautionary note, they publish a chart that is meant to guide physicians in their use of NSAIDs. The chart describes a “stepped approach” to prescribing painkillers. In this approach, the least dangerous (according to Heart Association information) should be prescribed before moving on to those drugs that are seen to carry greatest risk. The Heart Association chart can be found at Figure 7,

Studies seem to suggest that NSAID risk is dose dependent, though what a safe dose is for an individual has not been established. The risk to individuals from NSAIDs (available over the counter in many cases) is considered so serious that the U. S. FDA has mandated inclusion of black box warnings in their packaging. A description of this black box warning can be found at the NIH website; APPENDIX BLACK BOX WARNINGS OF INCLUDED DRUGS

As I repeat throughout my posts on this website: I am not a doctor or a researcher. I am a patient who reads and who seeks to understand. What I get from the research about NSAIDs is that these readily available drugs are serious medicine. The fact that they are over-the-counter and easily accessed belies the danger they may present to the medical consumer. Before taking any one of these, think for a moment. Be sure that the drug is important to your care. And, consult your doctor. Between the two of you, an informed decision about taking NSAIDs can be made.


Lupus and Sulfasalazine

By A. G. Moore 2/16/2013

DNA Sequencing
National Cancer Institute
Public Domain

While this essay specifically addresses the connection between sulfasalazine and lupus, the principles involved in the discussion have wider application. As an article in Pharmacological Review explains, “Individual variability in drug efficacy and drug safety is a major challenge in current clinical practice, drug development, and drug regulation” (Pharmacogenetics, Pharmacogenomics, and Individualized Medicine ). In other words, drug effectiveness varies according to the individual. The challenge is to find the most effective drug for each patient.

In the past, an appropriate patient/drug match was achieved through trial and error. Increasingly, though, science has improved the process of prescribing appropriate drugs for patients; often, a doctor can know in advance physical characteristics of patients which might make them more receptive to a drug or more likely to experience side effects.

Targeting medication to enhance effectiveness and avoid side effects is a gift to everyone: patients don’t lose precious, sometimes irreplaceable time with a therapy that doesn’t work; and dangerous, even lethal, side effects may be avoided.

Sulfasalazine is a drug therapy which can be administered today with greater confidence because of advances in genotyping–that is, identifying genetic elements which influence the way a patient processes medication. The history of this drug and its associated risks has been well-documented. In 1965 a physician from the Mayo Clinic reported that a patient he had been treating for inflammatory bowel disease went on to develop lupus. The doctor, Donato Alarcón-Segovia, attributed this development to the drug the patient had been taking: sulfasalazine. Since the 1965 case, sulfasalazine has been recognized as a drug which can induce lupus; the phenomenon is known as drug-induced lupus, or DIL. Sulfasalazine also may exacerbate disease in established lupus.

While doctors have known for a long time that drugs can induce lupus (first case was reported in 1945 in response to Sulfadiazine), this manifestation of lupus, known as drug-induced lupus or DIL, is usually described as being “mild”. However, “mild” lupus is not always the experience of a DIL patient. For example, an article which appears in Postgraduate Medical Journal (Postgrad Med J. 1982 February; 58(676): 98–99) describes a comatose 19-year old girl with “sulphasalazine-induced cerebral lupus erythematosus”.

Sulfasalazine has its uses: among other applications, some doctors find it a good alternative for controlling inflammatory bowel disease, rheumatoid arthritis, and, ironically, discoid lupus (see: Treatment of discoid lupus erythematosus with sulfasalazine: 11 cases ). According to the Lupus Foundation, sulfasalazine may even be prescribed to someone who has lupus if symptoms of rhuematoid arthritis are evident (see Lupus and Overlap).

So, what is a patient to do with this information? Whether sulfasalazine induces a “mild” or severe case of lupus, the development is obviously one a patient would like to avoid. As it turns out, this may be possible–without completely eliminating sulfasalazine from the physician’s list of viable treatment options. There is a way for doctors to profile individual patients and approximately assess their risk for developing sulfasalazine-induced lupus. Research has shown that almost all people who develop lupus after taking sulfasalazine fall in a group known as “slow acetylators” (see my blog: Medication Alert ). Those at high risk for developing sulfasalazine-induced lupus also tend to have specific genetic markers: HLA A1, HLA B8, and HLA DR3. If a patient does not have these markers and is not a “slow acetylator”, then the risk of developing drug-induced lupus may be greatly reduced.

Of course, it‘s impossible to tell how someone will respond to a drug. A serious discussion with the physician is in order before the decision is made to take sulfasalazine, or any medication. Risk and benefits need to be assessed.

Every drug has associated risks–not taking a drug also has associated risks. The more information a patient and doctor have about a proposed course of treatment, the more confidently can they assess the relative risk. In the case of sulfasalazine, it seems patients and doctors have the opportunity to weigh the medication’s benefits with more confidence than was available in the past.

Some articles helpful in learning about sulfasalazine and sulfasalazine-induced lupus:

Predisposing factors in sulphasalazine-induced lupus erythematosus from Rheumatology

Sulfasalazine-induced Lupus and Hypersensitivity, by Qingping Yao, and Bevra Hahn UCLA School of Medicine

Drug-induced lupus erythematosus on DermNet NZ

Update on the Management of Cutaneous Lupus Erythematosus, from the British Journal of Dermatology

Lupus Pancreatitis

By A. G. Moore 2/28/2013

CAT Scanner

by Liz West on Wikimedia Commons

Today’s blog was prompted by a question I read recently on Lupus UK.

Pancreatitis, whether related to lupus or not, is a very serious development and requires immediate medical attention. During an attack, the pancreas starts to digest itself because certain pancreatic enzymes have been released prematurely. These enzymes usually break down food in the small intestine but, since they have been put to work in the wrong place, they instead begin to break down the pancreas. The situation can quickly become life-threatening.

A doctor diagnoses pancreatitis by reviewing blood tests and abdominal scans. The reason the attack occurred, however, may not be easy to discover. Without knowing the cause, it will be difficult to design an appropriate treatment plan.

If pancreatitis is related to lupus, a specific protocol is recommended. In the medical journal Clinical and Developmental Immunology, outcomes for groups of patients with lupus pancreatitis are compared. Those patients who are treated promptly and aggressively fare far better than patients who do not receive this level of care. (See: Clinical and Developmental Immunology, Systemic Lupus Erythemtaosus-Related Acute Pancreatitis: A Cohort from South China.)

Pancreatitis occurs for a variety of reasons. In some people, the condition may be a result of lifestyle–excess consumption of alcohol is generally included in this category. For other people, medication may precipitate an attack. Azathioprine and steroids are often suspected antagonists in this scenario. Evidence about the link between pancreatitis and these two drugs is not uniformly persuasive; while azathioprine clearly may cause pancreatitis in some people, steroids are another matter. There is disagreement about whether steroid-induced pancreatitis actually exists.

Estimates vary about the risk of developing pancreatitis while on azathioprine. According to a 2003 study published in the American Journal of Gastroenterology, patients on azathioprine ran a 0.4% increased risk of developing pancreatitis. Another medical journal, Annals of Gastroenterology, reported that patients on azathioprine (or mercaptopurine, into which azathioprine is converted by the liver) who had inflammatory bowel disease stood a 3.1% increased risk of developing pancreatitis. So, if someone is on azathioprine therapy and pancreatitis develops, suspicion will first fall on the medicine as culprit. The drug will be discontinued and an alternate medication prescribed.

As for steroids and pancreatitis: there is growing disagreement about this relationship. The Lupus Foundation describes steroid-induced pancreatitis, and indicates that the cessation of steroid therapy is the remedy for this condition. However, a recent article in Clinical and Developmental Immunology states:Increasingly accumulated evidence showed that steroids do not trigger acute pancreatitis or cause increased mortality” in lupus patients. The lack of unanimity about the steroid/pancreatitis link increases the element of speculation in the diagnosis and treatment of pancreatitis.

When a situation exists where lupus has evidently caused pancreatitis, the way forward is clear for the doctor. High-dose steroids are the cure. This type of pancreatitis is called idiopathic lupus pancreatitis and is a very serious condition; it needs to be treated by experts in lupus care.

Since idiopathic lupus pancreatitis is thought to be rare, it can easily be overlooked by a physician who is not experienced in treating lupus. The complexity of treatment decisions facing a clinician increases if the lupus patient with pancreatitis is already on steroid therapy. Now the doctor has to figure out whether steroid therapy is appropriate or will make matters worse. The critical question has to be answered: Did steroids cause pancreatitis or will steroids cure pancreatitis?

An incident that occurred in a Slovenian hospital llustrates this point. A 33-year-old man evidently had pancreatitis. He also had lupus. Idiopathic lupus pancreatitis was suspected but high-dose steroid therapy did not elicit an improvement. Since the patient had been on steroid therapy, steroid-induced pancreatitis was suspected, but historically the use of steroids had not been a problem for this patient, so this theory fell out of favor. Finally, close examination of the many tests to which the patient had been subjected revealed that he had cytomegalovirus. It was this virus, the doctors concluded, which caused the pancreas to become inflamed. Treatment with Ganciclovir (an antiviral) was begun. The patient improved and eventually recovered.

The 33-year-old Slovenian patient obviously benefited from excellent medical care. The team of doctors who treated him were skilled and creative. They did not think in a box but used the latest research to problem-solve for their patient. Anyone with lupus who becomes ill wants just such a medical team in charge of their care.

Pancreatitis is a serious condition, for anyone. If you have lupus, the situation is even more perilous. If you do have lupus, know in advance who the best lupus doctors are and where the best lupus treatment facilities are located. When illness strikes, whether it’s pancreatitis or another ailment, be sure you are in a place that offers the best chance of a successful outcome.

Lupus: Signs and Tells

Lupus: Signs and Tells
By A. G. Moore 4/28/2013


Public Domain Wikimedia Commons

Sometimes I don’t take my own advice. Acknowledging this helps to keep me modest. Yesterday I received a strong dose of modesty as my husband’s predictions of impending trouble were realized. The truth of his insight became obvious during the night: my body confirmed what my husband had observed for several days, what he had insisted on before we turned in the night before. A flare was upon me. The thing about catching a flare early is that it’s usually easier to chase lupus away if the disease is caught on the upswing, before it’s taken hold.

Unfortunately, for my husband and others I love, signs of the flare included a compromise of my ordinarily good nature. Today, I must apologize to people–not for the first time–because I was irritable and rude. 

Then I’ll take my prednisone.

As everyone who has lupus knows, reluctance to begin an immune-suppressive regimen can seem reasonable. When lupus consists mostly of pain and lack of vigor, then taking medicine can seem self-indulgent. In my case, lupus has run a relatively mild course, so the temptation to ride out a storm without medicine is quite strong.

There are risks, though, to this laissez-faire approach. Lupus has, on occasion, turned on me and become vicious. Doctors have scratched their heads and alarm bells have gone off as I lay on a stretcher with symptoms that didn’t fit into any recognized pattern.

So I’m careful now, about guarding against vicious lupus episodes. I look for signs that tell me when a serious storm is brewing and I need to take action.

Signs of trouble for me do not always occur in a cluster. Some by themselves are compelling. For example, if I start to twitch–not just an eye or a jerk or two in my leg, but unremitting, sleep depriving, multi-site twitching–then I take action.  ‘Cause there’s trouble coming.

If my gut acts up and no amount of coddling will sooth it, I take action. ‘Cause there’s surely trouble coming and I never play chicken with when my gut is involved.

I don’t have lupus nephritis, which can be active without any signs such as those I just described.  Though some 40% of lupus patients present with nephritis early in the disease (first 5 years), about 5% of patients will develop kidney problems later. The material I cite below suggests that the best outcome for nephritis patients is realized with early diagnosis and treatment.  Since lupus nephritis can be silent, invisible, without early signs, it’s important that all lupus patients be monitored from time to time to see if this condition is developing.

What the literature says about treating early

There is little disagreement in the research community about the wisdom of getting early treatment when lupus attacks organs. To illustrate this cautious approach, I did some reading on two common manifestations of lupus. In both these cases, lupus nephrttis and GI lupus, early diagnosis and treatment are uniformly recommended. I am assuming we can extrapolate this cautious attitude to other forms of lupus where there is organ involvement.

Lupus Nephritis:
While not everyone with lupus has nephritis, this symptom is one of the first a doctor will look for when diagnosing the disease. The consensus about management of lupus nephritis is clear: early intervention saves lives and helps to prevent kidney failure.  In 2008, the American Association of Kidney Patients issued a report about pediatric lupus nephritis. The report concluded: “Early recognition of the pediatric patient…with prompt intervention…may prevent kidney failure…”.

In 2006, an article in the The Journal of Rheumatology concluded:”…early initiation of immune-suppressive treatment might be critical for the outcome of patients with lupus nephritis”.

And, finally, a 1999 article in the American Journal of Nephrology stated: “In most patients today, there is a gratifying response to early treatment…”


Gastrointestinal Lupus
The consensus about early treatment of lupus nephritis is mirrored in recommendations for treatment of gastrointestinal lupus.
Most sources suggest that GI lupus is often caused by lupus vasculitis, which is a very serious, life-threatening condition. However, there are aspects of lupus expressed in the GI tract which may not be caused by vasculitis. Two of these are pancreatitis and intestinal pseudoobstruction. Early treatment in any of these instances is essential.

A 2010 article in The World Journal of Gastroenterology described lupus mesenteric vasculitis, as “…a main cause of acute abdominal pain in SLE patients”. The authors of this posited that prompt diagnosis and “….appropriate intervention can avoid potentially fatal complications of…lupus mesenteric vasculitis”.

With regard to lupus pancreatitis, the same authors concluded: “As many as 57% of SLE-related acute pancreatitis cases may develop complications if not treated promptly.” And, finally, the authors discussed psuedo-obstruction. The recommendation for early treatment was unequivocal: “Early diagnosis and prompt treatment is critical to improve the overall outcome of SLE-related IPO (intestinal pseudo-obstruction) patients.”


As for my current lupus flare, this is small potatoes. I’m paying attention and treating early.  I am confident that this flare, like so many others, will subside quickly. Then my bottle of prednisone will be retired to a dark corner in the cabinet where it will wait until the next time one of my “signs” appears.