The Face of Sjogrens

What is Sjögren’s?

Sjögren’s (“SHOW-grins”) is a systemic autoimmune disease that affects the entire body. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and lymphomas.

Today, as many as four million Americans are living with this disease and nine out of ten patients are women with an average age of onset in the late 40’s. However, Sjögren’s can occur in all age groups, even in children.

Sjögren’s is not a “cookie cutter” disease & affects patients differently. Many patients experience dry eyes, dry mouth, fatigue and joint pain, but Sjögren’s also causes dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. And while some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning.

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.

Dr. Birnbaum for Sjogren’s Foundation

What is a Sjogren’s Flare?

Medical dictionaries define “flare” as a sudden exacerbation of a disease. A flare is different from the day-to- day variation of symptoms that patients with chronic diseases experience and is characterized as a large and rapid increase in a patient’s usual symptoms. I like to define a flare as a sudden and significant increase in the activity of a disease. This definition allows us to use quantitative measures of disease activity to compare levels of disease activity from one point in time (e.g. baseline) to another (e.g. flare).

Several measures of disease activity have been developed for Sjögren’s. The two most promising are the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI) and the European League Against Rheumatism Sjögren’s Syndrome Patient Reported Index (ESSPRI). The first is a tool that measures disease activity from the physician’s perspective in the many organs and systems affected by Sjögren’s.

The second measures disease activity from the patient’s perspective and includes a patient’s global assessment of disease and individual measures of dryness, pain, and fatigue. These surveys have been developed to consistently evaluate disease activity in research settings such as clinical trials. Nevertheless, they could be used in clinical practice as guidelines for evaluating disease activity in the office or clinic. The ESSPRI is a simple tool that could be used in the clinic, much like the use of the Health Assessment Questionnaire (HAQ) in patients with rheumatoid arthritis and other rheumatic diseases.

When patients say they are experiencing a flare, they usually mean that they are experiencing a marked increase in their Sjögren’s symptoms such as dryness of their eyes and/ or mouth, joint and muscle pain, and fatigue. Other symptoms might include swollen glands, skin rashes, or numbness and weakness in extremities. Physicians must make sure that these symptoms and signs are in fact a flare of the Sjögren’s and are not caused by other conditions that are not associated with Sjögren’s. These include infection, anemia, thyroid disease, drug side effects and fibromyalgia syndrome, to mention a few.

Since there is no specific treatment for Sjögren’s at present, treatment is symptomatic and dependent upon which organ system is involved. There are several things you can do to lessen the likelihood of getting a flare. Keep taking the medications prescribed for you on a regular, daily basis. Eat a healthy diet, exercise regularly and get restorative sleep. Try to minimize physical and emotional stress and develop good coping mechanisms when stress is unavoidable. Hydroxychloroquine has been shown to lower disease activity in systemic lupus erythematosus and may be similarly helpful in Sjögren’s. Your physician also might recommend other medications to improve your symptoms.

-Neil I. Stahl, MD, FACR

Top 10 Tips for Combating Gastroesophageal Reflux

 

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.  This can help neutralize stomach acid and reduce symptoms.

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.

Soo Kim Abboud, MD

The Sun & Sjögren’s: How to protect yourself

Sjögren’s patients, and those suffering from autoimmune disease in general, need to be cautious about their time in the sun. Ultraviolet (UV) radiation emitted from the sun and other light sources (such as some fluorescent lights) can alter immune function and lead to an autoimmune response in the body and skin.

In response to the sun, Sjögren’s patients can experience skin rashes, ocular sensitivity, pain, and disease flares. Sun sensitivity with Sjögren’s is associated with the autoantibody SSA/or Ro. Below are a few tips to help protect yourself this summer and year-round.

• Protect your skin and eyes through use of sunscreen, UV-protective lenses/sunglasses, ultraviolet light-protective clothing, hats, and non-fluorescent lighting. Sun-protective clothing is designed to protect your skin from UVA & UVB rays and is more reliable than sunscreen.
• Consider purchasing UV-protective car and home window tinting and films (which come in clear.)
• Wear sunscreen on areas not covered by sun-protective clothing, such as the neck and ears.
• Read sunscreen labels and look for the words “broad spectrum,” which protects from both UVA & UVB light. Note that the SPF ratings refer only to UVB rays.
• Use plenty of sunscreen with a higher number SPF. Most people only use about 1/3 the recommended amount of sunscreen. This reduces the benefit of the SPF rating.
• Remember to reapply sunscreen because water, humidity and sweating decrease sunscreen effectiveness.
• Investigate whether UV-protective clothing and eyewear, window shields, and sunscreens are eligible for reimbursement under your insurance plan or Flexible Health Care Spending Account.

Mona Z. Mofid, MD, FAAD

Sjogren’s Syndrome: Fatigue

Fatigue is one of the most prevalent and disabling symptoms of Sjögren’s syndrome. Here are some tips that can help

you cope with the problem:

Work with your doctor to find a specific cause and treatment for your fatigue. The possibilities may include systemic inflammation, poor sleep, fibromyalgia, depression, hypothyroidism, muscle inflammation or side-effects of medications.

Know your limits and pace yourself. Plan to do no more than one activity on your bad days. Try to do more on your good days, but don’t overdo it!

Listen to your body and plan to take a 20-minute time-out every few hours to help you get through your day.  Educate your friends and family about what you are going through and how the fatigue in Sjögren’s syndrome can come and go.

Develop a support system to help you with tasks. Ask friends and family members to be prepared to do one or two chores for you on your fatigue days. Give them specific instructions in advance and be reasonable with your expectations. Get at least eight hours of sleep every night. If you wake up at night, plan extra time for sleep.

Get your body moving every day! This may help not only your fatigue but also your chronic pain, poor sleep and depression. Start with five minutes of aerobic exercise daily (e.g. walking, biking, running, elliptical, treadmill) and increase the duration by an additional two-to-three minutes each month up to a maximum of 25 minutes daily.  If you have a heart or lung condition, consult your doctor first.

If you are still employed, ask your employer for accommodations because you have a medical condition. Try to work from home if possible to gain more flexibility with your work routine. Check the following resources (search“chronic fatigue”) to get more information on work accommodations and/or career options:

• Disability and Business Technical Assistance Center at http://www.dbtac.vcu.edu

• Job Accommodation Network at http://www.jan.wvu.edu

Identify the major stressors in your life and work with a mental health professional or your support system to minimize their impact.

Frederick Vivino, MD, FACR, University of Pennsylvania, P

What is a Sjogren’s Flare?

Medical dictionaries define “flare” as a sudden exacerbation of a disease. A flare is different from the day-to- day variation of symptoms that patients with chronic diseases experience and is characterized as a large and rapid increase in a patient’s usual symptoms. I like to define a flare as a sudden and significant increase in the activity of a disease. This definition allows us to use quantitative measures of disease activity to compare levels of disease activity from one point in time (e.g. baseline) to another (e.g. flare).

Several measures of disease activity have been developed for Sjögren’s. The two most promising are the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI) and the European League Against Rheumatism Sjögren’s Syndrome Patient Reported Index (ESSPRI). The first is a tool that measures disease activity from the physician’s perspective in the many organs and systems affected by Sjögren’s.

The second measures disease activity from the patient’s perspective and includes a patient’s global assessment of disease and individual measures of dryness, pain, and fatigue. These surveys have been developed to consistently evaluate disease activity in research settings such as clinical trials. Nevertheless, they could be used in clinical practice as guidelines for evaluating disease activity in the office or clinic. The ESSPRI is a simple tool that could be used in the clinic, much like the use of the Health Assessment Questionnaire (HAQ) in patients with rheumatoid arthritis and other rheumatic diseases.

When patients say they are experiencing a flare, they usually mean that they are experiencing a marked increase in their Sjögren’s symptoms such as dryness of their eyes and/ or mouth, joint and muscle pain, and fatigue. Other symptoms might include swollen glands, skin rashes, or numbness and weakness in extremities. Physicians must make sure that these symptoms and signs are in fact a flare of the Sjögren’s and are not caused by other conditions that are not associated with Sjögren’s. These include infection, anemia, thyroid disease, drug side effects and fibromyalgia syndrome, to mention a few.

Since there is no specific treatment for Sjögren’s at present, treatment is symptomatic and dependent upon which organ system is involved. There are several things you can do to lessen the likelihood of getting a flare. Keep taking the medications prescribed for you on a regular, daily basis. Eat a healthy diet, exercise regularly and get restorative sleep. Try to minimize physical and emotional stress and develop good coping mechanisms when stress is unavoidable. Hydroxychloroquine has been shown to lower disease activity in systemic lupus erythematosus and may be similarly helpful in Sjögren’s. Your physician also might recommend other medications to improve your symptoms.

-Neil I. Stahl, MD, FACR

 

Tips For Muscle and Joint Pain with Sjogren’s

 

Joint and muscle pain in Sjogren’s syndrome may result from a variety of causes including inflammation, fibromyalgia, age-related osteoarthritis, vitamin D deficiency, hypothyroidism etc.

Work with your rheumatologist to identify the specific cause(s) of your pain and find the best treatment regimen for you. Maintain a positive attitude and be an active partner in the management of your pain.
The tips below will also help:

• Become knowledgeable about your medications
  
• Get a good night’s sleep
  • Maintain a regular sleep schedule.
  • Set aside an hour before bedtime for relaxation. Listen to soothing music.
• Consider taking a warm bath before going to bed
  • Make your bedroom as quiet and comfortable as possible.
  • Avoid caffeine and alcohol late in the day.
  • Avoid long naps during the day.
• Exercise regularly with the goals of improving your overall fitness and keeping your joints moving, the muscles around your joints strong and your bones strong and healthy
  • A physical therapist, occupational therapist, or your health-care provider can prescribe an exercise regimen appropriate for your joint or muscle problem.
  • Start with a few exercises and slowly add more.
  • Make your exercise program enjoyable. Do it with your spouse or a friend. Include recreational activities, such as dancing, walking and miniature golf.
  • Try different forms of exercise, such as Tai chi, yoga and water aerobics.
• Balance rest and activity
  • Pace yourself during the day, alternating heavy and light activities and taking short breaks to rest.
• Control your weight
• Protect your joints and muscles
  • Use proper methods for bending, lifting, and reaching.
  • Use assisting devices, such as jar openers, reach extenders and kitchen and garden tools with large rubber grips that put less stress on affected joints.
• Use various therapeutic modalities that can relieve joint and muscle pain
  • Use heat (heating pads, warm shower or bath, paraffin wax) to relax your muscles and relieve joint stiffness.
  • Use cold packs to numb sore joints and muscles and reduce inflammation and swelling of a joint
  • Consider massage therapy.
  • Practice relaxation techniques, such as guided imagery, prayer and self-hypnosis.
• All of these suggestions are what most doctors tell us but they do work on all autoimmune diseases.
• sjogrens.org

13 Types of Sjogren’s Fatigue

Basic fatigue
1. This is the inherent fatigue that I attribute to the inflammatory, autoimmune nature of Sjögren’s. It’s with me all the time. It differs from normal fatigue in that you don’t have to do anything to deserve it. It can vary from day to day but is always there. For me, there appears to be a correlation between this kind of fatigue and sed rate (ESR). When one goes up, so does the other. I don’t know how often this phenomenon occurs. I could also call this my baseline fatigue, which fluctuates and gets better or worse. All of the following are superimposed on this basic fatigue.

Rebound fatigue
2. If I push myself too far and ignore the cues my body is sending me to stop and rest, my body will fight back. When I do more than I should, the result is an immobilizing fatigue. It comes on after the fact, i.e., do too much one day and feel it the next. If I push myself today, I very likely will have to cancel everything tomorrow. An extended period of doing more than I should will almost certainly cause a flare.

Sudden fatigue
3. This ‘crumple and fold’ phenomenon makes me resemble a piece of laundry. It comes on suddenly, and I have to stop whatever I’m doing and just sit down (as soon as I can). It can happen anywhere, at any time. It is the kind of fatigue that makes me shut off the computer in mid-sentence. It is visible to those who are observant and know what to look for, even though I make gargantuan efforts to disguise the fact that it is happening.

Weather related fatigue
4. Not everyone has this particular talent, but I can tell that the barometric pressure is dropping while the sky remains blue and cloudless. I feel a sweeping wave, a malaise, that sometimes lifts just after the rain or snow has started. Likewise, I know when a weather front is moving away, even while torrents of water are falling from the sky. I feel a lightening in my body and begin to have more energy. This kind of fatigue is accompanied by an increase in muscle aches and joint pain.

Molten lead phenomenon
5. This fatigue is present when I open my eyes in the morning and know that it is going to be a particularly bad day. It feels as if someone has poured molten lead in my head and on all my limbs while I slept. My muscles and joints hurt, and doing anything is like walking with heavy weights. It is often associated with increased symptoms of fibromyalgia and sometimes helped by heat and massage.

Tired-wired
6.Tired-wired is a feeling that comes from certain medications, such as prednisone, too much caffeine, or too much excitement or perhaps it is just a function of Sjögren’s. My body is tired but my mind wants to keep going and won’t let my body rest.

Flare-related fatigue
7. Flare-related fatigue is an unpredictable state of increased fatigue that can last for days or weeks. It may be caused by an increase in disease activity or an undetected infection. If the latter, it either resolves on its own, or eventually presents other signs and symptoms that can be diagnosed. Additional rest is essential to deal with this kind of fatigue, but rest alone will not necessarily improve it or make it go away. Once a flare begins, it is impossible to predict where it will go or how long it will last.

Fatigue induced by other physical conditions
8. Fatigue related to other physical causes, such as thyroid problems or anemia superimposed on Sjögren’s. This kind of fatigue makes you feel that you are climbing a steep hill when you are really walking on level ground. It resolves once the underlying organic condition is diagnosed and treated. Thyroid problems and anemia are both common among Sjögren’s patients, but many other kinds of fatigue may be superimposed.

Fatigue that impairs concentration
9. Fatigue that impairs concentration precludes thought, makes me too tired to talk, think or read. Fatigue robs me of memory and encloses me in a fog of cotton wool so thick I can’t find my way out until the fog miraculously lifts. For me, brain fog goes hand-in-hand with other kinds of pernicious fatigue.

Stress, distress, anxiety or depression
10. Stress, distress, anxiety or depression all can create a leaden kind of emotional fatigue that can be as exhausting as one due to physical causes. Although some people do not associate their increased fatigue with emotional states, many are aware of the effects of increased anxiety and depression, even if they cannot control what they feel. Intense emotion is very draining. Stress, anxiety and depression all are known to disrupt sleep.

Fatigue that comes from not sleeping well
11. Some people with Sjögren’s have trouble both getting to sleep and staying asleep. Some wake up in the morning feeling as if they had never slept at all. Many aspects of Sjögren’s affect sleep: being too dry, in too much pain or malaise; multiple trips to the bathroom, the need for water or to put in eye ointment all deter a good sleep. Lack of restorative sleep increases fatigue.

And two new ones:
Fatigue that comes with normal aging
12. I’m old enough for Medicare now and my friends are more tired too, although they seem to be able to do two or three or even four times what I can do on any given day. In fact, the gap between what they can do and what I can do just seems to be growing, despite my best efforts. It’s been a long time since I tried to keep up, but it still hurts that I can’t.

Chronic Illness Fatigue
13. Fatigue that comes from a chronic illness that just won’t quit. We’ve all heard the expression “sick and tired of being sick and tired” and that phrase truly captures what many of us feel. I would take it one step further. There’s a fatigue that comes with the uncertainty of a chronic disease. It’s a debilitating fatigue born of never knowing what will come next. The chronicity of Sjögren’s can wear me down and I have to make special attempts not to let it. When these attempts don’t work, I wait a while and try to find something else that distracts me from my illness.

It’s difficult to explain the unnatural quality and intensity of this fatigue to someone whose only experience has been with what is normal. We’re not talking about the same stuff. It’s apples and artichokes. Sjögren’s fatigue is pervasive. It assaults everything I do. There isn’t a part of my life that hasn’t been touched by it. It is there even on my happiest days.

Because people don’t understand, it’s often misinterpreted. “Is it depression?” a health care professional who didn’t know much about Sjögren’s asked me. I tensed. Was he saying it was all in my head? I began to get angry but then gave him the benefit of the doubt. I put my first reaction aside and decided he was trying to understand. I was relating something outside his frame of reference, and he was attempting to find a point with which he could identify.

When you describe Sjögren’s fatigue to someone who has never experienced it, you are asking him or her to think outside the box. You want them to understand an experience that is common to those who have Sjögren’s and many other autoimmune diseases but rare otherwise. Perhaps their first reaction will be negation or denial. While it’s always difficult to encounter expressions of disbelief, it is not uncommon.  I tried to see it as an opportunity to educate.

“No,” I said to the doctor who asked about depression, “think of it as a never-ending flu that varies in intensity but never goes away.” He grimaced.

Terri Rumpf Ph.d