Sjögren’s Syndrome: Pain Beyond Tears

People with Sjögren’s syndrome can develop hoarseness if their vocal cords become inflamed as part of the disease or become irritated from throat dryness or coughing.

If it was just diminished tears, Sjögren’s syndrome could be treated solely with eye drops.

HEALTH Similar to lupus, Sjögren’s (SHOW-grens) syndrome is a non-infectious, primarily hereditary and incurable autoimmune disease in which white blood cells called lymphocytes attack healthy tissue within the body. With Sjögren’s syndrome, the primary targets are moisture-producing lacrimal and salivary exocrine glands. Reduction in tears and saliva can affect vision, tooth decay, voice quality and the digestive system.

Systemic Effects of Moisture Reduction

Extraglandular involvement includes joint inflammation; particular forms of autoimmune thyroid, kidney, liver, lung, and skin disease; changes in peripheral nerve function; and a small percentage of patients may manifest a malignant lymphoma. [1] Sjögren-induced joint pain may feel like arthritis without the rheumatoid factor.

Rheumatoid factor is not only present in rheumatoid arthritis patients. It may be present in Sjogren’s syndrome patients or other autoimmune disease patients too. The same can be said for positive ANA tests. There are, however, specific antinuclear antibodies that occur commonly in Sjogren’s syndrome patients, though not all. The specific Sjogren’s syndrome antibodies are known as: anti-SS-A and anti-SS-B (also referred to as anti-Ro and anti-La respectively). [2]

People with Sjögren’s syndrome can develop hoarseness if their vocal cords become inflamed as part of the disease or become irritated from throat dryness or coughing. To prevent further strain on your vocal cords, try not to clear your throat before speaking. Instead, take a sip of water, chew sugar-free gum, or suck on sugar-free candy. Or else make an “h” sound, hum, or laugh to gently bring the vocal cords together so you can get sound out. Clearing your throat does the same thing, but it’s hard on the vocal cords, and you want to avoid irritating them further. [1]

Relieving Dry Eyes

Not all dry-eye discomfort is caused by Sjögren’s syndrome. Activities that minimize blinking or cause rapid evaporation, such as long-term computer use or exposure to wind, can result in dry-eye irritation. When Sjögren’s is the cause, the medical term is keratoconjunctivitis sicca; dry mouth is called xerostomia.

Dry eyes are helped by using artificial tears. Dry mouth can be relieved with saliva stimulants and mouth lubricants. Nonsteroidal anti-inflammatory drugs (NSAIDs) are used to manage joint pain. Corticosteroids may be prescribed to control inflammation if their is systemic (e.g., organs) involvement. Disease-modifying anti-rheumatic drugs (DMARDs) can be used to control an overactive immune system. [2]

Symptoms of Primary and Secondary Sjögren’s Syndrome

The absence of other autoimmune connective tissue diseases (notably rheumatoid arthritis or systemic lupus erythematosus), suggests Primary Sjögren’s Syndrome (PSS). Primary or secondary Sjögren’s syndrome can affect more than the lacrimal and salivary glands, causing the following symptoms [1]:

  • multiple sites of joint and muscle pain
  • prolonged dry skin
  • skin rashes on the extremities
  • chronic dry cough
  • vaginal dryness
  • numbness or tingling in the extremities
  • prolonged fatigue that interferes with daily life.

Neuropathy in Primary Sjögren’s Syndrome Patients

Extraglandular involvement of the peripheral nervous system may occur with reported frequencies from 10% to 60%. Peripheral nerve manifestations constitute sensory neuropathy, including sensory ganglioneuronopathy, sensorimotor, including polyradiculoneuropathy and demyelinating neuropathy, motor neuropathy, multiple mononeuropathy, trigeminal and other cranial neuropathies, autonomic neuropathy, and mixed patterns of neuropathy. Pathology in cases of sensory ganglioneuronopathy consists of loss of neuronal cell bodies and infiltration of T cells. Peripheral neuropathy in PSS often is refractory to treatment although newer biological agents may provide more effective treatment options. [3]

Though there is currently no cure for Sjögren’s syndrome, its symptoms can be managed. With more than 30 percent of patients presenting negative blood tests [4], physicians who routinely rely solely on ANA tests for diagnosis are doing their patients a disservice. This represents between 300,000 and 1 million persons in the U.S. whose complaints may be dismissed or untreated. Beyond standard blood tests, diagnosis can be based on Schirmer tests, slit-lamp examination, lip biopsy, salivary function tests, urine tests and chest x-ray. Don't ignore the symptoms.

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References
  1. Questions and Answers about Sjögren’s Syndrome, niams.nih.gov
  2. 10 Things You Should Know About Sjogren’s Syndrome, about.com
  3. Primary Sjögren’s syndrome associated neuropathy, Mellgren SI, et al, Can J Neurol Sci, 2007; 34(3);280-7, PubMed.org
  4. Sjögren’s Syndrome Foundation: Diagnosis, sjogrens.org