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Sjögren's Syndrome

and its Neurological Manifestations

by Emjay Rosales

     That itchy, burning feeling in the eyes, and desert-like feeling in the mouth. Have you ever felt this irritation? What if there is more to just dry eyes and dry mouth sensation?

WHAT IS SJÖGREN'S SYNDROME?

     It was in 1933 when the Swedish ophthalmologist Henrik Sjögren (pronounced as SHOW-grun) firstly described an autoimmune disease which involves salivary gland and tear gland inflammation. This is called the Sjögren’s Syndrome (SjS), an eponym linked to Sjögren.

 

     Autoimmune diseases happen when our immune system—which destroys the infectious agents of our body—attacks our body tissues. They produce abnormal production of antibodies in our autoimmunity which is likely to cause tissue inflammation. In SjS, the glands that produce saliva (salivary glands) and tears (lacrimal glands) to our body are inflamed and dysfunctional.

     Most patients with this condition are at age 40 or older, and more frequent in women. This is due to the influence of estrogen—the female sex hormone—to our immune system that put females at risk to autoimmune diseases.

HOW IT IS DEVELOPED

     When there is an absence of another rheumatic disorder or any connective tissue disease in the condition, like rheumatic arthritis or lupus, it is called the Primary Sjögren’s Syndrome (pSS). When it is associated with another rheumatic disorder, then it is called the Secondary Sjögren’s Syndrome.

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     Although there is still uncertainty why others develop SjS and others do not, and a need for scientific researches is imperative, scientists have established that SjS can be caused by genetic factors.  There might be triggering functions in certain genes that put a high risk on the condition.

SYMPTOMS

arthralgia

Symptoms of Sjögren’s Syndrome include:

 

  • dry eyes (xerophthalmia);

  • dry mouth (xerostomia);

  • dry skin (xerodermia);

  • the swelling of salivary glands (parotid gland enlargement);

  • joint pain (arthralgia);

  • muscle pain (myalgia);

  • difficulty swallowing;

  • burning throat;

  • persistent fatigue; and

  • vaginal dryness

RESEARCH STUDIES RELATED TO SJÖGREN'S SYNDROME

     In the October 2010 issue of the medical journal “Current Opinion in Neurology,” an article was published explaining that there are neurological manifestations in the pSS. In their findings, the most common neurological manifestation is the pure or predominantly sensory polyneuropathies which varies widely from ten to 60%. Sensory polyneuropathy is a condition when a damaged nerve disables an individual’s function to feel (sensation.)

     Another study is also published, this time by RMD Open, in their Volume 2 issued in April 2016. Entitled “Epidemiology of Neurological Manifestation in Sjögren’s Syndrome,” in this 15-center study, the clinicians examined 392 patients having dysfunctions in skin, joints, lungs, kidneys, muscles, vessels, and central nervous system (CNS) and peripheral nervous system (PNS).

 

     It is determined that the two anatomical components of our nervous system—the CNS and PNS—can be damaged and bring inflammation caused by SjS. In the study, 74 out of these 392 patients (18.9%) have neurological manifestations—63 of them have PNS manifestations (16%), and 14 have CNS manifestations (3.6%).

“The clinical and histological spectrum of neurological manifestations of Sjögren’s syndrome is becoming clear. The field needs further explanation of basic neuroimmunological mechanisms of neural injury, and controlled treatment trials.”

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—Department of Neurology

National Neuroscience Institute

Singapore

CNS AND PNS COMPLICATIONS

     One of the CNS complications is the myelitis or the inflammation of white and gray matter of the spinal cord. Because of some overlap with other symptoms, SjS patients might incorrectly receive diagnosis of Multiple Sclerosis, a disease that causes demyelination  of spinal nerves and brain cells (Taylor, D. 2017).

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     According to John Hopkins Neurology-Rheumatology professor Julius Birnbaum, M.D., neuropathy—inflammation to the peripheral nerves and one of the PNS complications—is under-diagnosed and under-treated in SjS because the syndrome may uniquely target nerves which are not tested on normal nerve-conduction tests, and there is a lack of any definitive blood tests.

 

      

TREATMENT

     “Many patients with Sjögren’s syndrome have autoantibodies, which bind to the body’s organ tissue and cells. However, more than 50% of patients with neurological manifestations of Sjögren’s may not have autoantibodies,” Birnbaum stated.

 

     Some people can manage the dry eyes and dry mouth irritation by treatments like using eyedrops, drinking water, and applying lotion. But for most SjS patients, there is a need for prescription. Doctors may prescribed pilocarpine and cevimeline which are drugs that can increase the saliva production. Arthritis medications may be suggested if the patient is having rheumatic disorders.

        These recent studies only show that considering the neurological signs of a syndrome like Sjögren’s is substantial and crucial as the foundation of the future research studies, and for the patients’ health.

 

        And for people having troubles with their health, do not wait for the worst case scenario.

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