This is Episode Eight of “Autoimmune Disease: Pieces of the Picture.” Dr. Beth Jonas tells listeners about Sjogren’s syndrome and how patients can manage the condition. Dr. Jonas is an Associate Professor of Medicine in the Division of Rheumatology, Allergy, and Immunology, and directs the UNC Rheumatology Fellowship Training Program. She is a rheumatologist with the UNC Thurston Arthritis Research Center.
“One of the things that we know is our saliva is full of immunoglobulin that really helps maintain healthy teeth, and when you lose that saliva, you’re at risk for dental decay. So keeping your teeth as clean as possible and visiting the dentist four times a year is very important for patients with Sjogren’s syndrome.”
– Beth Jonas, MD
Ron Falk, MD: Hello, and welcome to the Chair’s Corner from the Department of Medicine at the University of North Carolina.
This is our series where we explore topics related to autoimmune disease, to help patients and their loved ones understand and manage their condition. Today we will talk about Sjogren’s syndrome.
We welcome Dr. Beth Jonas, who is an Associate Professor of Medicine in our Division of Rheumatology, Allergy, and Immunology. Last week Dr. Jonas joined us for a discussion on rheumatoid arthritis. Welcome back.
Beth Jonas, MD: Thank you.
Sjogren’s & how it is diagnosed
Falk: I can barely say the word Sjogren’s. What does Sjogren’s syndrome mean? What is it?
Jonas: Sjogren’s syndrome is an autoimmune disorder that causes dryness of the eyes, dryness of the mouth, and sometimes enlargement of the parotid gland. Occasionally patients will have internal organ involvement where the disease involves the lungs or the kidneys but by far, most patients have what we call the sicca syndrome, which is dryness of the eyes and the mouth.
Falk: What separates patients who have dry eyes only from patients who have Sjogren’s? Some of them could have Sjogren’s, some of them probably don’t. How does one tell?
Jonas: That can be difficult sometimes. We see a lot of patients in our clinic who have dry eyes or dry mouth, or both. One of the things that can help us is certain antibodies that may be present in the blood, “Ro” and “La” antibodies. Most patients with Sjogren’s will have these antibodies in their blood, and sometimes we need to biopsy one of the salivary glands to make a definitive diagnosis if we’re not sure.
Falk: So if you took 100 patients who have dry eyes for example, how many of them will have Sjogren’s?
Jonas: I think it’s probably a pretty small number. I think it’s a minority of patients with dry eyes – there are so many things that can make your eyes dry. Medications can do it, aging can do it—many people will have dry eyes, but a very small percentage of them will actually have true Sjogren’s syndrome.
Falk: The same is probably true for dry mouth—lots of reasons to have a dry mouth. The combination of dry eyes and dry mouth—not being able to form spit, not being able to form tears, that combination, that at least should raise the possibility that this is Sjogren’s.
Jonas: That’s how patients come to us. When they have both of them, then it does raise your level of suspicion for Sjogren’s syndrome.
Falk: What helps in your diagnosis are blood tests, these autoantibodies that you just described. When do you do biopsies?
Jonas: I don’t biopsy most of the patients with Sjogren’s syndrome. If I have a high index of suspicion for Sjogren’s syndrome—someone has had profoundly dry eyes, profoundly dry mouth, they may have enlargement of the parotid glands or other lymphadenopathy, and if their antibodies are negative—which can happen—in those cases I might recommend a biopsy.
Falk: So you wouldn’t do a biopsy on everybody, right?
Jonas: No, most patients do not get a biopsy.
Treatment for Sjogren’s
Falk: What do you tell patients about treatment? Are there treatments for Sjogren’s?
Jonas: This can be very difficult. The treatment is really very symptomatic. If your eyes are dry, we often recommend certain kinds of wetting agents—artificial tears and other kinds of tear substitutes which can be used. The ophthalmologist may close off some of the tear ducts, to prevent the tears from moving out of the eye. There are other medications that have been approved for this, such as topical cyclosporine for the eyes, but the treatment is really symptom-oriented, and for the mouth also, using wetting agents–there are certain toothpastes, mouth washes, that can improve the moisture in the mouth.
Falk: So it’s really symptomatic treatment—you wouldn’t use immunosuppressive therapy unless the disease was in some other organ.
Jonas: Many patients with Sjogren’s syndrome will have either joint pain—arthralgia, or skin rashes, and under those circumstances we might use a medication called hydroxychloroquine which can be very helpful for those manifestations. Hydroxychloroquine really does not help the dryness at all, so if those other things are absent we generally do not use any systemic therapy.
Managing this condition
Falk: What kinds of factors can make Sjogren’s worse? What is the effect, for example, of stress?
Jonas: Well, like all autoimmune diseases, patients who feel stressed or are under unusual stress will often have exacerbation of their symptoms. Learning to manage that stress in your life is very important.
Falk: And exercise is fine?
Jonas: Exercise is great.
Falk: Really it’s just trying to make the best of a difficult situation, especially if you have very dry eyes and a very dry mouth.
Jonas: Right, and I tell my patients it’s very important to have an ophthalmologist that they work closely with, one they see regularly and can help them with that, and also to visit their dentist, usually four times a year for a good cleaning. One of the things that we know is our saliva is full of immunoglobulin that really helps maintain healthy teeth, and when you’ve lost that saliva, you’re at risk for dental decay. So keeping your teeth as clean as possible and visiting the dentist four times a year, is very important for patients with Sjogren’s syndrome.
Falk: Generally patients can learn for themselves how best to use these wetting agents, how best to figure out ways to make sure there’s enough moisture in the mouth. If they can manage that, their long-term outlook is pretty good.
Jonas: That’s true, and that learning curve is really important for patients to learn how to manage their symptoms with the agents that we have, but the prognosis and the outlook for the future for most patients with Sjogren’s syndrome is actually quite good.
Risk for lymphoma
Falk: One of the more worrisome, albeit uncommon concerns for patients with Sjogren’s syndrome is that they can have a form of a cancer that affects the lymphoid tissue, and that’s called a lymphoma. Lymphomas can occur in patients with Sjogren’s. How do you screen for lymphomas and what do you tell patients? When they come to see you, when do you start worrying about whether a lymphoma is present, and the next steps?
Jonas: First, I want to say that the incidence of lymphoma in patients with Sjogren’s syndrome is actually pretty low. Probably 5% of patients or less with Sjogren’s syndrome will ever develop a lymphoma. So the vast majority of patients will never have to worry about this.
If you’re going to get a lymphoma, it usually occurs more than 6, 7, or 8 years after the diagnosis, so it’s a late finding. When I evaluate patients I’m looking for swollen lymph glands, new fevers or other clues that might tell us that lymphoma may be occurring. We know that patients who have persistent enlargement of the parotid glands are at particular risk, so we look for that all the time and we risk-stratify our patients. There are some blood tests that might help us to predict which patients are at higher risk, but as I mentioned, the risk is actually pretty low.
Falk: Dr. Jonas, where are the parotid glands? Could you describe it for patients, where they should be feeling?
Jonas: A parotid gland is right in front of the ear, sort of on the jaw line. You may know the parotid glands from when you had the mumps as a kid, so-called chipmunk cheeks. They’re right in front of the ear and just on the jaw line.
Falk: That’s the gland that is involved in Sjogren’s syndrome. If a patient’s looking for more information or trying to research Sjogren’s syndrome, where should they look?
Jonas: There are a couple of places to look. The American College of Rheumatology, www.rheumatology.org, the Arthritis Foundation, www.arthritis.org, and there’s also a Sjogren’s Syndrome Foundation which has excellent information on the web, www.sjogrens.org.
Falk: Thank you, Dr. Jonas. And thanks to our listeners for tuning in. If you enjoy this series, you can subscribe to the Chair’s Corner on iTunes or like the UNC Department of Medicine on FaceBook. Stay tuned for our next episode.
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