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.
Keep Shopping for Sjögren’s!
When using the link below, 40% of every Tupperware purchase will be donated back to the SSF. Stock up on great items knowing that your purchase is helping support the SSF’s life changing initiatives.
Click Here to Start Shopping
Together we will conquer Sjögren’s! Promotion ends November 19th.
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.
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:
July is Dry Eye Awareness Month! The Sjögren’s Syndrome Foundation partners with various companies during July to help educate the public about dry eye symptoms, treatment options, and the possible cause being Sjögren’s. We hope you enjoy our July blogs aimed to promote dry eye awareness and education.
Q) Many eye drops claim to have disappearing preservatives. Are these the equal of preservative- free drops, or should they still be used like eye drops with standard preservatives?
A) The development of “disappearing preservatives” has allowed eye drops to be formulated in multi-use dropper bottles for convenience without the risk of surface damage that can occur with the more potent and persistent preservatives. The mechanism by which such new preservatives “disappear” is usually due to chemical changes in the preservative that occur upon exposure to air or the tear film. The most common such chemical reaction is oxidation of the preservative, turning it into an inactive molecule. It must be remembered, nevertheless, that the inactive molecule can be something to which sensitive patients may react. It is worthwhile, therefore, that the patient be alert to any intolerance of such medication which can occur as irritation, discomfort or red eyes. The “disappearing preservative” eye drops can be used up to four times a day in most cases without difficulty and some patients can use them even more frequently than drops with regular preservatives. It should be remembered that other eye drops, particularly those used to treat glaucoma, can contain preservatives as well and, therefore, it is important for patients to keep track of how many drops are being instilled in the eye during the day.
Truly preservative-free eye drops contain no such preservative chemicals but, therefore, require special packaging that limits the amount of the solution in the dropper to usually only one or two drops. The challenges of the smaller packaging can be a nuisance, but if the patient is sensitive to even the “disappearing preservative” this nuisance can be worth the better tolerance to the lubricant.
-Gary N. Foulks, MD
Thank you to our Dry Eye Awareness Month Partner:
While the exact reasons are unknown, many patients with Sjögren’s suffer from gastroesophageal reﬂux disease (GERD). This can cause a wide variety of symptoms that can be mistaken for other conditions. Symptoms may include persistent heartburn and/or regurgitation of acid, stomach pain, hoarseness or voice change, throat pain, sore throat, difﬁculty swallowing, sensation of having a lump in the throat, frequent throat clearing and chronic cough (especially at night time or upon awakening).
Tips for combating gastroesophageal reﬂux in the throat:
1. Avoid lying ﬂat during sleep. Elevate the head of your bed using blocks or by placing a styrofoam wedge under the mattress. Do not rely on pillows as these may only raise the head but not the esophagus.
2. Don’t gorge yourself at mealtime. Eat smaller more frequent meals and one large meal.
3. Avoid bedtime snacks and eat meals at least three-four hours before lying down.
4. Lose any excess weight.
5. Avoid spicy, acidic or fatty foods including citrus fruits or juices, tomato-based products, peppermint, chocolate, and alcohol.
6. Limit your intake of caffeine including coffee, tea and colas.
7. Stop smoking.
8. Don’t exercise within one-two hours after eating.
9. Promote saliva ﬂow by chewing gum, sucking on lozenges or taking prescription medications
such as pilocarpine (Salagen®) and cevimeline (Evoxac®). This can help neutralize stomach acid and reduce symptoms. Check the SSF's Product Directory (free of charge to all SSF members) to see the products available.
10. Consult your doctor if you have heartburn or take antacids more than three times per week. A variety of OTC and prescription medications can help but should only be taken with medical supervision.
The SSF thanks Soo Kim Abboud, MD for authoring this Reﬂux and Your Throat Patient Education Sheet. Dr. Abboud is an Assistant Professor with the Department of Otolaryngology, Head and Neck Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.