Sjögren’s is a systemic autoimmune disease. Normally, the immune system works to keep your body healthy — for example, by killing germs or healing wounds. But with a systemic autoimmune disease like Sjögren’s, something goes wrong with the immune system that causes it to harm the body’s own organs and tissues. Researchers, too, are investigating ways in which the nervous system interfaces with the immune system and lead to the development of Sjögren’s and many of its symptoms.
Everyone with the disease experiences Sjogren’s differently. It can affect any part of the body — including the nervous system. When Sjögren’s impacts the nervous system, it can cause a wide range of symptoms. Many people with Sjögren’s report experiencing at least some neurological (having to do with the nervous system) symptoms. And in some cases, neurological symptoms can be one of the first signs of Sjögren’s disease.
About the Nervous System
The nervous system is a complex body system that’s made up of 2 main parts:
- The central nervous system (CNS) is the brain and spinal cord (a large bundle of nerves that runs along the backbone). The brain sends out commands to the rest of the body, and the spinal cord carries information to and from the brain.
- The peripheral nervous system (PNS) is all the nerves that branch out from the spinal cord and run throughout the rest of your body. The peripheral nerves and the brain send information back and forth via the spinal cord, and the PNS carries out commands coming from the brain.
The 2 systems work together to send messages, signals, and information throughout the body. The nervous system controls or impacts many important body processes — including things like thinking, breathing, moving, digestion, seeing, hearing, and feeling.
The PNS is made up of 2 subsystems — the autonomic nervous system (ANS) and the somatic nervous system (SNS). The ANS helps to regulate (control) body processes you don’t have to think about — like breathing, making your heart beat, digesting food, or regulating your body temperature. The SNS helps to guide your movements by carrying signals from the CNS to your muscles.
How Sjögren’s Disease Affects the Nervous System
Sjögren’s disease can affect any part of the nervous system. Symptoms can be different depending on which part of the nervous system is impacted.
Experts aren’t yet sure exactly how many people with Sjögren’s have neurological symptoms — and they’re still working to understand all the ways that Sjögren’s can affect the nervous system.
Disease mechanisms are biological processes that cause a disease to happen (or get worse). There are a few possible mechanisms that scientists think may cause neurological symptoms in Sjögren’s:
- Vasculitis: This happens when the immune system attacks or damages the body’s blood vessels. This means that parts of the nervous system, such as the brain, might not be getting enough blood flow.
- Inflammation: Inflammation is when the body releases certain chemicals to respond to a problem in the body, like an injury or infection. But when the immune system isn’t working properly — like in an autoimmune disease like Sjögren’s — it might produce inflammation when it isn’t needed. Over time, this can damage your body’s healthy tissues, including your nerves.
- Problems with the glymphatic system: The glymphatic system is like the brain’s waste management system. It moves a fluid called cerebrospinal fluid (CSF) throughout the brain to clean it and keep it working properly. The glymphatic system is only active during sleep, so it’s possible that having sleep problems (which are common in Sjögren’s patients) can impact how well it's working.
- Problems with the blood-brain barrier: The blood-brain barrier is a membrane inside the blood vessels inside your brain. It helps to protect your brain by keeping germs, toxins, and other harmful substances being carried through the blood vessels from getting into your brain tissue. Scientists think that inflammation can damage the blood brain barrier.
Keep in mind that these aren’t the only possible disease mechanisms that may cause Sjögren’s to affect the nervous system. There’s still plenty that experts don’t know about how Sjögren’s disease works.
Neurological Signs and Symptoms of Sjögren’s Disease
There’s a wide range of neurological signs and symptoms that are associated with Sjögren’s disease. Many patients report that they started experiencing neurological symptoms long before other well-known Sjögren’s symptoms, like dry eye or dry mouth.
If Sjögren’s affects the CNS, it can cause:
- Headaches or migraines
- Aseptic meningitis: This is when the membranes around the brain and spinal cord (the meninges) are inflamed. It can cause headache, stiff neck, fever, light sensitivity, or fatigue (feeling extremely tired).
- Myelopathy: This affects the spinal cord, which is part of the central nervous system (CNS). It occurs when the spinal cord is damaged due to compression, inflammation, or reduced blood flow (called ischemia). Symptoms may include weakness, numbness, coordination problems, or difficulty with balance. Myelopathy often worsens over time if not treated.
- Encephalopathy: This is a general term for when the brain isn’t working as it should. Some symptoms linked to encephalopathy include having “brain fog” (trouble remembering or concentrating), confusion, seizures, difficulty speaking or understanding language.
- Cognitive impairment: This is trouble thinking, remembering, or paying attention. Some research has shown that cognitive impairment is worse when Sjögren’s disease activity is high.
If Sjögren’s affects the PNS, it can cause:
- Peripheral neuropathy: This is a general term for when your peripheral nerves are damaged or don’t work as they should. There are many different types of peripheral neuropathies. Depending on which nerves are involved, symptoms can include motor symptoms (trouble moving, muscle weakness or paralysis, or uncontrolled movements), sensory symptoms (tingling, numbness, clumsiness or trouble balancing, and pain), or autonomic symptoms (like problems with your blood pressure, sweating too much or not enough, bladder or bowel issues, or trouble with sex).
- Cranial neuropathies: This can also occur as part of peripheral neuropathy. Cranial neuropathies is when a cranial nerve (any of the 12 main nerves that run from the brainstem to the head, neck, or trunk) is damaged or isn’t working properly. They affect the cranial nerves, which control sensation and movement in the face, head, and neck. One example is trigeminal neuropathy, which affects a cranial nerve responsible for facial sensation. It can cause facial numbness, tingling, or sharp, shooting pain in the face or jaw.
- Small fiber neuropathy: This is a type of peripheral neuropathy that’s been identified in some people with Sjögren’s disease. The most common symptoms of small fiber neuropathy are having a tingly or “pins-and-needles feeling” in your hands or feet or legs and arms, numbness, or a change in sensation in the extremities (known as a “stocking and glove sensation”, as if normal feeling is dulled by clothing). This type of neuropathy can sometimes be painful or described as “burning”. When small nerve fibers are damaged, symptoms of autonomic nervous system dysfunction can occur. These might include heart palpitations (when it feels like the heart is pounding or skipping beats) or not being able to feel pain or temperature changes on a small part of your body.
- Radiculopathy: This affects the nerve roots as they exit the spinal cord and is considered part of the peripheral nervous system (PNS). It occurs when a nerve root is compressed or irritated. Symptoms may include pain, numbness, tingling, or weakness that travels along the affected nerve. Symptoms can improve over time, sometimes with little or no treatment.
- Ataxia: This symptom is when movement of the body is uncoordinated. The muscles, sensory nerves, and brain can all be involved in ataxia. People with ataxia may be unsteady when walking, have trouble with fine motor skills (using the hands for precise tasks, like buttoning a shirt), be clumsy, or have trouble speaking clearly.
- Dysautonomia: This is a broad term for when the ANS — the part of the PNS that controls body processes you don’t have to think about — isn’t working as it should. It can cause a wide range of symptoms, including dizziness or lightheadedness (such as after you stand up from lying down), brain fog, problems digesting food, and problems with the glands that produce moisture (like the tear ducts or salivary glands).
Keep in mind that there’s some overlap between the CNS and PNS when it comes to symptoms. For example, both problems with the CNS and the PNS can cause pain or brain fog. That means it’s not always easy to tell exactly what’s causing a neurological symptom.
If you notice any neurological signs or symptoms, talk with your doctor right away. Some nervous system problems can be serious — so it’s important for your doctor to evaluate your symptoms and determine exactly what’s causing them. That way, you can get treatment if you need it.
Managing Neurological Signs and Symptoms of Sjögren’s Disease
Neurological signs and symptoms of Sjögren’s can have a big impact on your day-to-day life and how you feel — and they can be challenging to manage.
If you have neurological symptoms, you can:
- Share them with your doctor. Be sure to talk with your primary care doctor, rheumatologist (a doctor who treats conditions like Sjögren’s), or a neurologist (a doctor who specializes in nervous system health) about any neurological symptoms you have — especially if you have symptoms that are new, change over time, or make it difficult to do normal things, like walking, speaking, or sleeping.
- Ask for a referral to see a neurologist. If you’re not already seeing a neurologist, consider asking your current doctor if they can refer you. A neurologist is an expert in diagnosing and managing neurological problems.
- Keep track of your symptoms with a symptoms log. Write down information like when a symptom started, how long it lasted, and how much it bothered you. It’s a good idea to make note of what you were doing or how you were feeling before a symptom started too. Share the log with your doctor at your appointments.
- Prioritize healthy habits. You can’t always prevent neurological problems from happening. But taking steps to protect your nervous system can help you feel better and make existing symptoms easier to manage. Healthy habits to promote nervous system health include getting enough sleep, limiting alcohol, and staying hydrated.
Depending on the symptoms you’re experiencing, your doctor might recommend certain tests to learn more about your condition. For example, they may suggest doing an MRI (a type of imaging test) to check your brain or spinal cord or a sleep study (when you spend a night in a special sleep lab to help diagnose sleep problems).
Treatment for neurological symptoms also depends on your specific symptoms. Some treatments are aimed at improving your symptoms or making them easier to manage. Other treatments focus on controlling the immune system to stop it from damaging the nervous system further.
Get Support for Managing Sjögren’s Disease
If you’re experiencing neurological symptoms of Sjögren’s disease, know that you’re not alone. When Sjögren’s affects the nervous system, it can have a big impact on your daily life.
Get support in managing neurological symptoms of Sjögren’s — or other Sjögren’s symptoms — from your doctor or a neurologist.