Should I treat my Sjögren’s differently if I have primary vs secondary Sjögren’s?
The terms "Primary and "Secondary" were first used in the 1960s and were devised by researchers who wanted to distinguish between those Sjögren’s patients whose disease was not "complicated" by other major rheumatic or autoimmune disorders. When carrying out research, the investigators wanted to make sure they were looking at purely Sjögren’s patients and not looking at outcomes for patients who had, for example, both Sjögren’s and rheumatoid arthritis (RA). They thought that could confuse clinical trials looking at how well a specific therapy worked. As such things usually happen, though, the terms began to carry over to general clinical diagnosis and medical discussions and thought patterns, and that's where everything became complicated and not helpful for patients and not even helpful for the clinicians treating them. It didn't always make a major difference for the research, either.
So, first, what do the terms mean? "Primary" has been defined as a Sjögren’s patient who does not have another major rheumatic and/or autoimmune disease and "Secondary" as a Sjögren’s patient who does. But as you can imagine, it's not always simple or easily apparent. If a patient has another major rheumatic, autoimmune disease such as lupus, RA, scleroderma or the autoimmune disease multiple sclerosis, they would have traditionally been categorized as have "Secondary Sjögren’s." The term "Secondary" has not been applied to the prevalent autoimmune thyroid diseases, however, which are common in Sjögren’s, and so the terms are somewhat tricky.
Also, investigators have confronted a dilemma when a patient has had Sjögren’s for many years and been labeled as "Primary" and then is diagnosed with another major rheumatic and/or autoimmune disease and automatically being re-labeled as "Secondary Sjögren’s." And to complicate matters more, some clinicians have now started saying their patient has "Primary Sjögren’s” and "Secondary lupus"(for example) while others undiagnosed the patient from having "Primary Sjögren’s” and changed the diagnosis to "Secondary Sjögren’s." How confusing!
Does it really matter? NO - It certainly doesn't matter to the patient or the clinician treating a patient. It doesn't alter treatment, since treatment for these diseases is based largely on the clinical manifestations and symptoms. All patients should be treated on a case-by-case basis.
Does the label mean your disease is more or less severe? ABSOLUTELY NOT. If someone has labeled you as having "Secondary Sjögren’s," it does not mean that your Sjögren’s is less severe or secondary in importance to the other condition. It also doesn't mean that symptoms that were labeled as Sjögren’s symptoms previously are now symptoms of the other disease. Autoimmune diseases often overlap, and sometimes it's difficult to tell if a symptom is Sjögren’s or, say, lupus. In fact, Sjögren’s is the most frequent disorder that occurs in conjunction with other autoimmune and rheumatic diseases, so, again, your signs and symptoms must guide the treatment.
Does the label make a difference as to whether patients are monitored for specific complications or not? NO. Again, your management and treatment should depend on your manifestations of autoimmune disease. You might be labeled as having lupus AND Sjögren’s or rheumatoid arthritis AND Sjögren’s, and then your symptoms and diseases should be managed according to your specific case and with complications specific to each in mind.
Traditionally, it has mattered to an investigator running clinical trials, but even that is now being called into question. First, diagnosis and pigeon-holing these diseases is not always easy or an exact science. Second, investigators didn't mind if patients with RA or lupus who entered clinical trials also had Sjögren’s and thought it did not muddy the results of trying a new therapeutic. Why? Because, again, like clinical treatment, the trials were primarily targeted toward clinical manifestations - for example, joint pain, which can occur in several rheumatic diseases including Sjögren’s. While a few manifestations might be distinctive of one disease versus another, such as the joint damage that occurs only in rheumatoid arthritis, the results still were based on the manifestation.
The Sjögren’s Syndrome Foundation is trying to move the medical and scientific community away from these terms, because they usually are NOT helpful or necessary. In fact, they are most often used out of habit, and while we recognize that habits can be hard to change and it can take a long time for a majority to start using different terminology, the SSF is on a mission to accomplish this. Simply put, someone either has Sjögren’s or does not have Sjögren’s. Having another identifiable disease doesn't change the fact that the patient has Sjögren’s, and a somewhat arbitrary decision about which additional diseases and conditions might change a patient between "Primary" and "Secondary" no longer makes sense.
-Katherine Hammitt, SSF Vice President of Research
This article was first published in The Moisture Seekers, SSF's member newsletter.
There are many different types of neuropathies in Sjögren’s. These neuropathies can have different causes and may require different diagnostic techniques & therapeutic strategies. Unlike other autoimmune disorders, in which the neuropathies predominantly cause weakness, the neuropathies in Sjögren’s primarily affect sensation and can cause severe pain.
Recognition of unique patterns & causes of neuropathies in Sjögren’s is important in arriving at appropriate therapies.
Top 10 Peripheral Neuropathy & Sjögren’s Facts:
1. Recognize that neuropathic pain is a chronic disease. Just as most causes of neuropathies and neuropathic pain in Sjögren’s do not come on suddenly, reduction of neuropathic pain can take a while.
2. Initial and predominant neuropathies in Sjögren’s can occur anywhere in the feet, thighs, hands, arms, torso and/or face.
3. Many different symptomatic therapies for neuropathic pain are available. Both physician and patient awareness of potential benefits and side-effects can help tailor an appropriate approach.
4. While the class of tricyclic anti-depressants (TCAs) often constitutes a first-line tier of therapy in other neuropathy syndromes, the TCAs can increase mouth and eye dryness and therefore are not routinely used as front-line therapies in most Sjögren’s patients.
5. Electrophysiologic tests may help in the diagnosis of neuropathies affecting larger nerves which are coated by an insulator called myelin. However, neuropathies affecting smaller-fiber nerves that lack this myelin coating cannot be detected with these tests.
6. Special diagnostic tests, including the technique of superficial, punch skin biopsies (small biopsies of three millimeters and not requiring any stitches), can help in the diagnosis.
7. A relatively rare neuropathy can cause significant weakness in Sjögren’s patients. In contrast to other neuropathies which develop slowly, this neuropathy can present with very abrupt-onset of weakness. This so-called “mononeuritis multiplex” occurs because the blood-flow through vessels which nourishes nerves is suddenly compromised.
8. In general, immunosuppressive medications are almost always warranted to treat “mononeuritis multiplex” neuropathy. In contrast, the role of immunosuppressives is not well-established in other neuropathies, including neuropathies that cause pain but are not associated with weakness.
9. Sjögren’s patients frequently wonder whether pain associated with a neuropathy means they are at an increased risk for more severe motor weakness. While there are exceptions, if weakness is not present at onset, it most likely will not occur.
10. Neuropathic pain can be alleviated and assuaged, although there may initially be a “trial-and-error” process with different and perhaps multiple agents.
The information from this post, provided by rheumatologist and neurologist Dr. Birnbaum, was first published in The Moisture Seekers, SSF's member newsletter.
Your Support is Making a Difference for Sjögren’s!
SSF Fall Update & Ways to Help
It's been a busy and exciting fall here at the Sjögren's Syndrome Foundation! In addition to the SSF's fall community awareness events, the Foundation staff is now getting ready to attend another scientific meeting, the American College of Rheumatology's (ACR) Annual Meeting, to focus on professional education of Sjögren's.
At the ACR Annual Meeting, the SSF will be both an exhibitor and presenter, in addition to holding working groups for those who treat Sjögren's. This meeting is the SSF's biggest venue for reaching rheumatologists and providing them the educational materials needed to better identify and treat their Sjögren's patients.
These SSF initiatives and programs that further our mission are only possible because of your support. As you can see below, there are many different ways that you can help this fall. I encourage you to learn about these opportunities and join our fight to conquer Sjögren's!
On behalf of the SSF, thank you for sharing your story, telling others about the SSF, attending our events, volunteering your time, donating and helping create a future of hope for all Sjögren's patients.
Chief Executive Office
New T-Shirt Design for Team Sjögren’s Turkey Trot Kits!
Learn what was Turkey Trot Alumni Erin's favorite part of joining Team Sjögren's!
My family and I wore our Team Sjögren’s T-Shirts and as we were racing, multiple people would comment about their friends, family or themselves being affected by this disease. Knowing that the Turkey Trot was bringing people together for a cause I believed in was a wonderful feeling.
Click Here to read the rest of Erin's Story
Whether you run or not, if you are a patient or know someone with this disease, we are all part of Team Sjögren's.
It's CFC Time!
We hope you will consider the Sjögren's Syndrome Foundation when choosing where to allocate your Combined Federal Campaign donation this year!
The SSF's CFC Code is: 10603
Tell your co-workers, friends and family how important it is to choose and write in the SSF on their campaign forms too! Every dollar impacts Sjögren's! For more information, please contact the SSF at 1-800-475-6473.
Save the Date:
SSF National Patient Conference, April 17-18, 2015
The Grand Hyatt Tampa Bay, Florida
2900 Bayport Dr,
Tampa, FL 336078
The SSF's annual National Patient Conferences are the best way to learn more about Sjögren's. Over the years, these Conferences have helped thousands of patients and their family members gain a better understanding of Sjögren's while giving them an opportunity to meet fellow Sjögren's patients.
If you want to be your own best patient advocate by gaining a thorough understanding of all the key aspects of Sjögren's, then our National Patient Conferences are for you!
More Details Coming Soon
Upcoming SSF Events
November 8, 2014- Nashville Area Sjögren's Awareness Walkabout
November 15, 2014- Streams in the Desert Trivia in Glen Ellyn, IL
November 15, 2014- Capital Region Sjögren's Awareness Walkabout
November 16, 2014- Boston Sip for Sjogren's
January 9-11, 2015- Team Sjogren's Disney
February 20, 2015- Phoenix Sjogren's Walkabout & Health Fair
April 17-18, 2015- SSF National Patient Conference
Dryness from Sjögren’s may affect any organ in the body that secretes moisture, because the body’s immune system is mistakenly attacking its own moisture-producing glands. Almost every Sjögren’s patient experiences some degree of dry mouth, which is caused by a decrease in both the quantity and quality of saliva.
Saliva plays an immensely important role in the oral cavity, which is why proper dry mouth care and attention is critical for a person’s overall oral health. If dry mouth persists for months or years, the decreased salivation can lead to many oral complications such as severe and progressive tooth decay, oral infections (particularly fungal), difficulty swallowing, or a combinations of these. Dr. Rhodus explains more below about the importance of saliva:
"A human being normally produces approximately 1.5 liters of saliva per day. There is a typical diurnal circadian rhythm in the production of that saliva with one peak in the mid-morning followed by a relative decrease until the second peak occurs around early evening.
Saliva flow normally is decreased at night. Saliva is produced by several glands: the submandibular glands (which lie bilaterally just under the posterior jaw) produce most of the quantity of saliva (45%) and it is a mixed fluid with both mucous (thick, stringy fluid) and water but containing most of the proteins; the paired parotid glands (which are in the mid-face just in front of the ears) produce primarily serous (or watery) fluid and accounts for about 35% of the total quantity; the sublingual glands (again in a pair just beneath the anterior tongue) are much smaller and contribute only about 10% of the total volume; and finally there are hundreds of small minor salivary glands in the lips, palate and throat which contribute a relatively small, but important portion of natural salivary flow.
The normal quantity of saliva naturally provides necessary oral lubrication and moisture to assure comfort and function for the individual, but saliva does much more than that. At least equally as important as this volume of saliva, if not more so, is the composition of saliva, which is rich in constituents which have potent digestive, coating, protective, antimicrobial, antiacid, lubricative and homeostatic properties. Saliva is much more than water. In fact, saliva contains approximately 60 important, protective constituents including: immunoglobulins,electrolytes, buffers, antimicrobial enzymes, digestive enzymes and many others, all of which make saliva an essential contributor to the health and homeostasis of the oral cavity. This is the reason that water or artificial salivas are a poor substitute; none of them have the rich composition of ones own natural saliva."
This post is the introduction from "The Importance of Silvia" article written by Nelson L. Rhodus, DMD, MPH, FICD, that was first published in The Moisture Seekers, SSF's member newsletter. SSF members can click here to read the full article on the members section of the website.
It is not unusual for some illnesses involving chronic pain to take years to find an accurate diagnosis. Patients may see a dozen or more doctors while seeking help, answers and relief. Some may have multiple medical appointments in the span of just one week. They may see different specialists for different symptoms, as if body parts function independently of one another. The patient may be on many medications, coping with side effects that can be brutal, and too many of these services focus on what the patient cannot do with little or no attention paid to what they can do. The very process can leave the patient feeling more helpless, more depressed, more fatigued, more stressed. How frustrating must it be to have the very things you do to get better and regain control of your life make you worse?
If you or someone you know is one of these patients, here are some tried-and-true ideas that have helped others that you also may find helpful:
- First, do not settle for bad medicine. Acknowledging that these cases do not fit well into today’s quick medical model, if you do not feel heard or helped, find another doctor. If that doctor does not meet your needs, find another doctor. Bear in mind that cheapest in the short run may end up being the most expensive long term if you are not getting good results. There are many good, skilled and caring doctors, but it may take some time to find the right one for you. The physician who is willing to be your partner and your educator and treat you with dignity and respect is the right choice for you.
- Be your own advocate. No one knows your body better than you. No one knows your pain better than you. No one knows what makes you happy better than you. You are the expert on you.
- Resist buying into the idea that our medical system is so broken good treatment is not available. I will never debate the idea that the system is broken. I will debate the idea that good treatments are not available. It may require defining and redefining what constitutes “good treatment” as you figure out what works best for you, but you will know it when you find it.
- Just because a treatment may be considered “holistic” does not mean it does not have value. Just because something is approved by your insurance company does not mean it does have value. Neither comes with any guarantee and both should be met with healthy skepticism. Leaving any positive option out of the mix is a missed opportunity.
- Remember to pay as much attention to your mental health as you do your physical health. The mind-body connection is real and plays a major role in combating any illness.
- Consider limiting the number of medical appointments you have in one week, if at all possible. Too much focus on what ails you can bring down the best mood and invite in the boogeyman at 3am with dark thoughts that never helped anyone. Too many appointments also can eat up time that would have been available for a yoga class, a trip to the gym, or lunch with a good friend. Balance is important.
- I know you’ve heard this one before: You are what you eat. A deprivation diet is not necessary or helpful or sustainable, but a healthy diet filled with a rainbow of foods that are good for all of us are even more important for those with special needs. Consider including a qualified nutritionist in your treatment team. Click here to view SSF suggested nutritional resource available for purchase, "The Immune System Recovery Plan"
- Take a relationship inventory. If you have people around you who drag you down, who think they know what is best for you better than you and your doctors, or who may even question the reality of your illness, it’s time to clean house. The drain of toxic people and toxic relationships sucks away energy better used in creating your best possible life.
- Whatever you love doing, do it - and then, do it again!
This article by Darlene Cross, MS, MFT, was first printed in the March 2013 issue of The Moisture Seeker, SSF's patient newsletter for SSF members.
Is there any speculation as to what’s driving this big upswing in the diagnosis of men and children with Sjögren’s?
My gut feeling is that it has to do with the improvements in awareness and medical and dental education in recent years. The Sjögren’s Syndrome Foundation (SSF) has spent years trying to train the physicians and nurse practitioners about how prevalent and serious the disease is. We finally have a celebrity who unfortunately was diagnosed with Sjögren’s and although nobody likes to see somebody become ill, it has done a lot to help the entire public realize how serious it is, particularly the idea that people look a lot better than they feel and that it may take years to diagnose it unless you take the symptoms seriously.
I can tell you at the University of Pennsylvania, where I work, the oldest medical school in the United States, we only started giving our first Sjögren’s lecture to the first year medical students about four years ago. And that was only after years of me fighting with the curriculum committee to get it included in the rheumatology course for the first year students. So, we’ve made a lot of progress and I think that’s an example of the benefits of all this work.
-Frederick B. Vivino, MD, MS, FACR
This "Question & Answers" article was first printed in the The Moisture Seeker, SSF's patient newsletter for members.
I am honored that on July 1, I was introduced as the new Chairman of the Sjögren’s Syndrome Foundation Board of Directors. My wife, Anne, and I became passionate volunteers for the SSF when Anne was diagnosed with Sjögren’s, and I have seen first-hand what it’s like to live with this devastating chronic disease.
The SSF is an inspiring organization and I am personally excited, as well as honored, to be leading us over the next two years! I hope you will join me, and also consider making a donation, as the SSF embarks on a two-year journey to intensely focus on changing the way Sjögren’s is treated, managed and monitored. We can't do this without you.
The past few years have seen amazing progress in awareness and research for Sjögren's. At the last American College of Rheumatology (ACR) Annual Meeting, the premier scientific and clinical meeting for rheumatologists in the United States, more abstracts and presentations specific to Sjögren's were on the program than ever before. The SSF annual educational luncheon, held for rheumatologists during ACR, was standing room only and we had more rheumatologists visit our exhibit booth requesting complimentary patient brochures for their offices than at any previous ACR meeting.
Additionally, during ACR, late breaking news was announced that six genes specific for Sjögren’s were identified by SSF-supported research at the Oklahoma Medical Research Foundation. This identification is the first time that genes have been connected solely to Sjögren's and marks the beginning of unraveling the genetic basis for Sjögren’s. This research was a huge breakthrough and could lead to determining a patient’s individual risk of certain complications and developing future treatment.
That is why today I am asking for your support. The SSF needs your help to expand our efforts to educate healthcare professionals, increase clinical trials for new therapies and to develop better diagnostics. Just imagine what these accomplishments would do for the quality of life of a Sjögren’s patient - a matter very close to my heart.
As a proud husband of a Sjögren’s patient, I see the struggles that my wife experiences each and every day and I want nothing more than for her disease to be nonexistent. The critical work of the SSF brings us HOPE for the future as they lead the charge to improve the lives of all patients.
Thank you in advance for your gift as we strive to keep our momentum alive and impact the future of Sjögren’s.
Chairman, SSF Board of Directors
Plaquenil, hydroxychloroquine (HCQ), is an anti-malarial medication that has been proven to be useful in the treatment of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and other inflammatory and autoimmune diseases. In Sjögren’s, Plaquenil is used to treat many symptoms of Sjögren’s including fatigue, joint symptoms of arthritis and arthralgias (joint pain), dry mouth and dry eyes. Similar to its use in systemic lupus erythematosus, many clinicians feel that it is useful in reducing general Sjögren’s “disease activity.”
One of the reasons that physicians feel comfortable in prescribing Plaquenil is its low risk to benefit ratio. This means that the side effects of Plaquenil are mild and infrequent compared with its potential benefits. As with any medication, allergic reactions including skin rashes and non-allergic reactions can occur. The side effect that is of greatest concern is retinal toxicity.
Retinal toxicity of Plaquenil may manifest itself with subtle disturbances of the retinal pigment epithelium which may eventually lead to complete destruction of the macula in the form of bull’s-eye maculopathy.
Several risk factors may increase the likelihood of retinal toxicity from Plaquenil such as, age of greater than 60 years, daily dose more than 6.5 mg/kg; use of the drug more than 5 years, obesity, preexisting retinal disease and, renal or liver failure. Early detection of the maculopathy is of critical importance to discontinue Plaquenil in order to stop or slow retinal damage. Unfortunately, clinically evident early structural changes can be subtle and usually preceded by abnormalities in functional tests such as visual field examination, multifocal electroretinography (mfERG), fundus autofluorescence (FA) imaging, and optical coherence tomography.
Recent findings suggest that Plaquenil toxicity can develop among patients that are taking the drug at a daily dose lower than the suggested “safe” dose and/or have been on Plaquenil for shorter than five years. Unfortunately, cessation of Plaquenil intake may not be a remedy since not infrequently, patients will develop objective evidence of progression despite discontinuation of the drug. Thus, the possibility of toxicity should not be disregarded and close monitoring of the ocular findings is required.
As a precaution, patients treated with Plaquenil are advised to get a baseline eye exam prior to starting the drug and then annually thereafter
This information provided by Neil I. Stahl, MD & Tongalp H. Tezel, MD was first printed in the The Moisture Seeker, SSF's patient newsletter for members.
Sicca is a word derived from the Latin siccus, meaning “dry.” Dryness of the exocrine glands, particularly the eyes and mouth, is referred to as “sicca syndrome” or “sicca complex” when there is no evidence of autoimmune disease present. While sicca symptoms occur in the vast majority of Sjögren’s patients, not everyone with these symptoms has Sjögren’s. Because of this, it is important to establish an autoimmune cause for the dryness. Sometimes other causes may be found, such as radiation therapy to the head, certain medications, or Hepatitis C or HIV infections. If no cause is found, the patient should be followed carefully for possible Sjögren’s because it sometimes takes years for the diagnosis to become clear.
Dryness from Sjögren’s may affect any organ in the body that secretes moisture. In addition to changing the quantity and quality of saliva and tears, dryness may manifest in the airways, nasal passages, sinuses, throat, skin, and in women, the vagina. Some Sjögren’s patients initially present with recurrent sinus infections, severe vaginal dryness, chronic dry cough, and so on. All types of specialists, not just eye doctors and dentists, need to keep Sjögren’s in mind as a diagnostic possibility, especially when dryness is severe, persistent, or accompanied by systemic symptoms such as fatigue and widespread muscle and joint pain. Dryness can be quite serious, causing dental disease, eye pain and even visual impairment. However, these issues should not detract from the often missed point that Sjögren’s is much more than sicca syndrome. Sjögren’s is a serious systemic autoimmune disease that can affect almost any organ in the body.
-Sarah Schafer, MD
This information was first printed in the April issue of The Moisture Seeker, SSF's patient newsletter for members.
As anyone with Sjögren’s knows, many things can exacerbate the discomfort of dryness, while there are other factors that can either soothe the dryness or advance a condition of moisture that can prevent it.
Here are things you can do on a day-to-day basis that can alleviate your symptoms and help you feel and look better.
- Do Exercise
Regular exercise unquestionably does all sorts of good things for us. The main medical benefit is perhaps the power to decrease inflammation, which it does through the release of endorphins. For that reason, exercise contributes to the health of the ocular surface. Regular exercise- at least 20 minutes of exercise that increases your heart rate 5x a week- is highly recommended for dry eye sufferers.
- Do Take Showers
A hot bath can be a relaxing indulgence, but the steam tends to rise away from you. It's much better to be upright in a shower, with the steam coming at you constantly. Moreover, whether you intend it or not, water from the shower head or bouncing off your body, splatters into your eyes and literally cleans them out.
- Do Catch some Zzzzzzs
I cannot emphasize enough how important getting as much sleep as possible is to mitigating the discomfort of dry eye. A deep sleep, replenishes the tear film and soothes the ocular surface.
- Do Drink Water
You should drink 6-8 glasses of water a day. That's water- plain and simple- not sodas, sugary juices or artificially flavored drinks. Water is needed by all of the body's organs- by the skin, the kidneys, the liver, the heart and the eyes as well.
- Do Keep up with Friends & Family
There is increasing evidence that social interaction is as good for us as exercise, a good night's sleep or eating natural food. It is also a fact that the smile you wear while you're happy with friends can actually reduce the exposure of the ocular surface.
- Don't get Stressed
Stress can affect many other factors that have a direct impact on dry eye: sleep, your blink rate, and even what you eat. All of that leads to the kind of inflammation that can exacerbate a range of ailments, including a dry eye disorder. There are many different kinds of stress and there are many ways to manage it. Find the way that works for you, and learn as best you can to keep stress at a minimum.
- Don't work your eyes too long
Perhaps the most important thing to avoid if you suffer from dry eye is a long stretch of consecutive visual tasking. Whether it's working at a computer, watching television or reading- break up the time you spend doing it.
- Don't Smoke, Drink Alcohol or Caffeine
Smoke, alcohol and caffeine all dehydrate the body, including the eyes. Be aware of what these activities are doing to your dry eye, and try to reduce the frequency or eliminate all three if you can.
This information is provided by Robert Latkany, MD
Author of "The Dry Eye Remedy" and Founder & Director of the Dry Eye Clinic at the New York Eye & Ear Infirmary
Thank you to our Dry Eye Awareness Month Partner: