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The Link Between Neuropathy and Sjögren’s Syndrome
By Marc Treihaft, MD, FAAN

EDITOR'S NOTE: Peripheral neuropathy refers to any disease of the peripheral nervous system. It is characterized by a spectrum of symptoms and signs that reflect nerve damage and dysfunction. In recognizing the broad scope of this disease, this article is part of an ongoing series of articles "Recognizing the Broad Scope of Neuropathy" that draw attention to conditions that include peripheral neuropathy as a symptom.

Sjögren's syndrome is a chronic autoimmune disorder affecting more than 4 million Americans. First identified in 1933 by Swedish ophthalmologist, Dr. Henrik Sjögren, the disorder is defined by its two most common symptoms—dry eyes and a dry mouth.

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In Sjögren’s syndrome, the body’s immune system attacks the tear and salivary glands—resulting in decreased production of tears and saliva. Sometimes, other tissues can also be affected. Patients usually report dry, gritty or burning sensation in the eyes, dry mouth, difficulty talking, chewing or swallowing, a sore or cracked tongue, dry or burning throat, dry or peeling lips, a change in taste or smell and increased dental decay. When the peripheral nerves are involved, patients present with sensory neuropathy characterized by pain in the arms and legs, sometimes with unsteadiness and incoordination. Less frequently, patients can present with multifocal neuropathy characterized by weakness and sensory loss in various parts of the body.

Sjögren’s syndrome should be suspected in anyone with a sensory or multifocal neuropathy, especially if it is associated with dry eyes or mouth. The Schirmer test, which consists of placing strips of paper in the lower eyelid, can reveal reduced tear production. The use of rose Bengal dye can detect areas of the cornea and the conjunctiva (the membrane covering the white of the eye) that have been damaged by the dryness. Blood tests frequently show elevated antibodies— anti-SSA/Ro or SSB-La. The diagnosis can also be made with a salivary gland biopsy, showing inflammatory changes.

Treatment is directed at suppressing the underlying inflammation and ameliorating the painful symptoms. Although there are no controlled trials that test the efficacy of any particular treatment, improvements have been reported after treatment with corticosteroids, intravenous gammaglobulin (IVIG), rituximab, cyclophosphamide or other anti-inflammatory agents.

Dr. Marc Treihaft is Medical Director , CNI Neuromuscular and Peripheral Nerve Disorders Center and Professor of Neurology, University of Colorado Health Sciences Center.

 

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