You’ve been waiting for this appointment for months. Your mind races as you wait for your name to be called. This all started when you made an appointment with your doctor on your dentist’s recommendations. You had more cavities than usual and your dentist recommended you get checked for an autoimmune condition. An autoimmune condition? Really? Sure, you have been tired the last few months, but life is busy and who isn’t tired these days? Your doctor did some tests and said you have rheumatoid arthritis. WHAT? You are given a copy of your labs, the name of rheumatologist and told to take Advil.
The four-month wait for the rheumatology appointment gives you plenty of time to investigate your labs, and to your dismay, you find you are ANA positive. Dr. Google immediately informs you that you likely have lupus. Let the spiraling commence. Why didn’t my doctor mention this? How could this happen? Am I going to die?
They call your name and usher you back. Blood pressure is a little elevated – not surprising given what you are bracing yourself to hear. Waiting again. Finally, the doctor arrives. She looks over your labs, does a physical, and says “You don’t have lupus or RA.” A wave of relief washes over you. Your future suddenly becomes clear again. “But you have Sjogren’s Syndrome.”
Excuse me? Show-grins? What the f@#k is that?
This story, or some version of it, I have found is how many people come to find out they have Sjogren’s.
What is Sjogren’s Syndrome?
Like many rheumatic diseases, it is a systemic autoimmune condition. “Autoimmune” means the immune system, for reasons that are unclear, attacks the cells and tissues of your own body. “Systemic” simply means it affects the entire body. The typical Sjogren’s patient is a woman over 40 years old. Certainly men and children can get it too, but, by far and away, it is a condition of middle-aged women. Although it is systemic, the most commonly affected organs are the lacrimal and salivary glands. The lacrimal glands, located around the eyes, produce tears and the salivary glands produce saliva. Autoimmunity from Sjogren’s leads to inflammation in these glands which then results in gland malfunction. Malfunctioning lacrimal or salivary glands leads to dry eyes and dry mouth. This is problematic as our tears are necessary for our eye heath. Tears provide lubrication that prevents corneal scratches and infections. Similarly, our saliva keeps our gums and teeth healthy and free of cavities.
But it is systemic, remember? There must be other symptoms, right?
Right. Many of the non-glandular symptoms of Sjogren’s can be very similar to lupus and rheumatoid arthritis. Fatigue, brain fog, skin rashes, and photosensitivity are frequent. Joint pain is very common, although not all Sjogren’s joint pain is the same. Some experience a fibromyalgia – type pain, characterized by muscular pain, with possible numbness and tingling in the area. Other patients get an inflammatory joint pain, like RA, manifested by large, swollen, and tender joints. Not as common, but still important, Sjogren’s patients can develop problems of their lungs, kidneys, and nervous system. Thankfully these complications are infrequent but are still important to keep in mind. This makes it critical to keep in touch with you rheumatologist when you have Sjogren’s syndrome.
How your doctor categorizes Sjogren’s Syndrome.
Doctors think of Sjogren’s as either primary or secondary. Sjogrens oftentimes travels with other autoimmune conditions, such as rheumatoid arthritis, scleroderma, lupus, or myositis. When this occurs, we refer to it as secondary Sjogren’s, as it is “secondary” to another autoimmune condition. But Sjogren’s syndrome can also develop by itself. In those cases, we refer to it as primary Sjogren’s. When given a diagnosis of Sjogren’s, knowing if it is primary or secondary is important. Not because the way we approach it differs (because it does not), but because when we know this distinction we can be confident other conditions have been investigated.
Recommended questions for your doctor:
Is my Sjogren’s primary or secondary? And what tests have you done to confirm this?
And what other tests can we do to rule out any other autoimmune condition?
Why is Sjogren’s so confusing?
And why are so many given a diagnosis of rheumatoid arthritis or lupus initially?
We must consider several factors to make a diagnosis of Sjogren’s. Like every other autoimmune condition, the diagnosis relies on more than just labs. When concerned about autoimmunity, doctors will usually test for the ANA and the rheumatoid factor (RF). But there’s a twist. Both the ANA and the RF are often positive in patients with Sjogren’s syndrome. This makes doing further testing, specifically looking for Sjogren’s syndrome, critical. Two antibodies should be checked: the anti-SSA antibody and anti-SSB antibody. These antibodies (either one or both) are positive in 70-80% of those with Sjogren’s. However, this leaves 20 – 30% of Sjogren’s patients without antibodies, which makes things even more confusing! And what if you are in that 20 – 30%? At this point, a further step is usually necessary. This involves obtaining tissue proof that there’s inflammation around the glands. with a lip biopsy, or a minor salivary gland biopsy. This is done by an ENT (Ears Nose & Throat) specialist.
Must know! Sjogren’s + Lymphoma
Finally, Sjogren’s patients need to be aware of the close association between Sjogren’s syndrome and lymphoma. Lymphoma can show itself in many ways, but often appears as very large, hard, non-movable lymph nodes. These can develop under the jawline, under the armpit, or in the groin. In general, swollen lymph nodes are actually very common and most of the time do not indicate lymphoma. A small, tender, or movable lymph node is usually a normal lymph node and doesn’t need to cause concern. This risk of lymphoma warrants, at least, annual or semi-annual checkups for all Sjogren’s patients.
Millions of people can live full lives with Sjogren’s syndrome. Understanding what it is, how it may or may not be related to conditions such as rheumatoid arthritis and lupus, and the possible complications enable Sjogren’s patients to take full control of their bodies and their condition.