April is Sjögren's Awareness Month, which seems like a good time to cover the basics of one of the more common and most underdiagnosed autoimmune conditions I treat.
What is Sjögren's — and how do you say it?
First things first: it's pronounced "Show-grens." The condition is named after Henrik Sjögren, a Swedish ophthalmologist who first described it in the early 20th century.
Sjögren's is an autoimmune condition in which the immune system primarily attacks the body's moisture-producing glands, most notably the salivary glands and lacrimal glands (responsible for tear production). It can occur on its own, which we call primary Sjögren's, or alongside another autoimmune condition like rheumatoid arthritis or lupus, which is called secondary Sjögren's.
It's one of the more common autoimmune conditions and tends to affect women in their 40s and 50s, though it can occur at any age. The condition is significantly underdiagnosed and many patients go years before getting the right answer.
Symptoms
The hallmark symptoms are dry eyes and dry mouth, but Sjögren's is often more than that. Fatigue, joint pain, brain fog, and swollen lymph nodes or salivary glands are common. In more severe cases, patients can develop nerve damage, lung involvement, and vasculitis (inflammation of blood vessels). There is also a modestly increased risk of lymphoma in patients with Sjögren's, which is one reason we follow these patients carefully over time.
Because dry eyes and dry mouth have many causes (medications being a big one), Sjögren's often gets missed or attributed to something else. Antihistamines, certain antidepressants, and nerve pain medications are among the common culprits for dryness symptoms, and these always need to be considered before landing on a diagnosis.
Diagnosis
There is no single test that confirms Sjögren's. Diagnosis requires a careful clinical evaluation, a detailed history and a thorough exam. Most patients will have a positive ANA and positive SSA and/or SSB antibodies (also called anti-Ro and anti-La). However, seronegative cases exist, meaning negative antibodies do not rule out the diagnosis. This is one of the reasons Sjögren's can take a long time to diagnose.
When clinical suspicion is high and labs are negative, we can pursue other testing such as a minor salivary gland biopsy, which looks for characteristic inflammatory changes in gland tissue. Musculoskeletal ultrasound of the salivary glands is another option, though less specific. Other supportive lab findings include elevated immunoglobulins, elevated ESR, positive rheumatoid factor, and low complement levels (C3/C4).
Management
Sjögren's is a chronic condition and there is currently no cure. Management is largely focused on controlling symptoms and preventing complications.
For dry eyes, lubricating eye drops are a mainstay. Preservative-free formulations are preferred, and drops containing carboxymethylcellulose or sodium hyaluronate tend to work better than simple saline. Gel drops at night can help with overnight dryness. Prescription options like cyclosporine (Restasis) and lifitegrast (Xiidra) are available, though they can take several months to reach full effect. I often recommend patients discuss punctal plugs with their eye doctor as well. Think of it as plugging the drain. By blocking the tear duct, you hold on to the tears you have longer rather than losing them. Regular ophthalmology follow-up, at least twice a year, is important.
For dry mouth, there are medications that stimulate residual saliva production. Pilocarpine (Salagen) and cevimeline (Evoxac) are the most commonly used. They can be helpful but do come with side effects including sweating, flushing, GI cramping, and frequent urination, which limits tolerability for some patients. Saliva substitutes like Biotene products provide temporary relief but wear off quickly. I still recommend them because saliva plays an important role in dental health and cavity prevention. Along the same lines, I recommend prescription fluoride toothpaste (such as Prevident) and dental visits every three to six months. Diet matters too. Avoiding sugary and acidic foods and drinks, staying hydrated, using a humidifier, and minimizing medications that worsen dryness when possible all make a difference.
For systemic symptoms, hydroxychloroquine is often used. It is not particularly helpful for dryness itself, but I use it most for patients with inflammatory joint pain. The data on fatigue is mixed, though it is reasonable to try given its favorable side effect profile.
A note on pregnancy
For women with Sjögren's who are pregnant or planning to become pregnant, the presence of SSA antibodies is something we take seriously. These antibodies can cross the placenta and in a small number of cases cause fetal heart block — a condition affecting the baby's cardiac conduction system, and it's permanent. The overall risk in a first pregnancy is approximately 1 to 2%, which is low but not trivial given the severity of the condition. That risk rises significantly (to around 16 to 18%) if a prior pregnancy was already affected. Hydroxychloroquine is often recommended to reduce this risk. I refer all of my SSA-positive patients to maternal fetal medicine for closer monitoring during pregnancy.
There is also a risk of neonatal lupus, which can cause a temporary rash and low blood counts in the newborn. This is generally less concerning than heart block and tends to resolve over six to eight months as the mother's antibodies clear from the baby's system.
What's coming
The treatment landscape for Sjögren's is evolving. There are several promising therapies in clinical trials targeting the underlying disease process rather than just managing symptoms. We will do a deeper dive on those when and if they reach approval. Stay tuned.
Summary
Sjögren's is more common than most people realize and still frequently goes unrecognized for years. If you have persistent dry eyes and dry mouth alongside fatigue, joint pain, or brain fog, it is worth bringing up with your doctor. An evaluation by a rheumatologist can help sort out whether Sjögren's might be part of the picture.