Sensory Neuropathy and Sjögren’s Syndrome

Sensory neuropathy and Sjögren's syndrome are two distinct health conditions that are closely linked. There is a high proportion of patients who are affected by both conditions, suggesting that there may be some association in their pathophysiology, or that one may be causative of the other.

In most cases, the symptoms of sensory neuronopathy present prior to the symptoms of Sjögren's syndrome, although this does not necessarily correlate to the order in which the conditions begin in the body.

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What is sensory neuropathy?

Sensory neuropathy, also known as ganglionopathy, is a health condition involving the degradation of the neurons in peripheral nervous system (PNS). In particular, the dorsal root ganglia are affected in this condition.

Sensory neuropathy can result in distinct symptoms, such as the inability to control voluntary muscle movement as usual and an abnormal gait.

What is Sjögren's syndrome?

Sjögren's syndrome is an uncommon systemic autoimmune disease that affects approximately 1% of the population. The characteristic symptoms of the syndrome include dry eyes and dry mouth, among other varied signs and symptoms.

Damage to the PNS has been observed in many patients with Sjögren's syndrome and is estimated to affect up to 50% of patients with this syndrome. There are several different conditions of the PNS that are related to Sjögren's syndrome, one of which is sensory neuropathy.

Overview of Sjogren’s Syndrome

Symptoms and presentation

In most cases, the initial symptoms of sensory neuropathy are recognized before the symptoms of Sjögren's syndrome. For this reason, a neurologist is often involved in the diagnosis of the concurrent form of the conditions, which is typically around 3-5 years after the initial diagnosis.

Patients will initially notice signs of changes in their voluntary muscle movement and an abnormal gait, both of which are characteristic of sensory neuropathy. Some time afterward, other symptoms linked to Sjögren's syndrome develop, such as the dry mouth and dry eyes.

For most patients with Sjögren's syndrome-related sensory neuropathy, the disturbances to the sensory system are unilateral and asymmetric. The limbs are affected to the greatest extent, particularly the arms; however, the trunk and face may also be involved.

Most individuals with sensory neuropathy and Sjögren's syndrome are elderly, with the mean age of onset at 64.9 years.

Pathological association

The high prevalence of Sjögren's syndrome-associated sensory neuropathy suggests a link in the pathology of the conditions. Some medical researchers have indicated that Sjögren's syndrome may, in fact, be involved with causing sensory neuronopathy.

While only 21% of patients with both conditions having antibodies for Sjögren's syndrome at the initial consultation, 43% have been found to develop positive serology tests after approximately seven years. These findings alone show the need to consider the involvement of the syndrome during the diagnostic process of neurological disorders, such as sensory neuronopathy.

Other associated diseases

Sjögren's syndrome is not the only health condition linked to sensory neuronopathy and thus may not be the underlying cause of the condition for all patients who exhibit signs of the syndrome.

For this reason, it is important to consider all possible causes for the condition and evaluate the individual's overall health status. This involves screening for diabetes mellitus, vitamin B12 deficiency, and monoclonal gammopathy.

Other characteristic features may also have a role to play in the development of this disease including chronic overconsumption of alcohol, human immunodeficiency virus (HIV), amyloidosis, and paraneoplastic syndromes.

References

Further Reading

Last Updated: Nov 8, 2022

Yolanda Smith

Written by

Yolanda Smith

Yolanda graduated with a Bachelor of Pharmacy at the University of South Australia and has experience working in both Australia and Italy. She is passionate about how medicine, diet and lifestyle affect our health and enjoys helping people understand this. In her spare time she loves to explore the world and learn about new cultures and languages.

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Comments

  1. Dolly Stewart Dolly Stewart United States says:

    My hands, fingers feel "funny,"  I was diagnosed with sensory ganglionopathy ataxia and Lupus. Any suggestions for pain management. Thanks!!

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