Primary Sjogren’s Syndrome

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What is PSS?

This chronic autoimmune disease is characterized by inflammation that affects exocrine glands (think salivary glands, lacrimal glands, etc) and leads to dryness phenomena. Commonly, patients who have primary sjogren’s syndrome (pSS) are very tired. The severity, degree of fatigue, is influenced by pain, depression, and sleep disturbances.

Fatigue is a hard and vague term. At times, we are all tired! We could define fatigue as an overwhelming sense of tiredness, lack of energy, feeling of exhaustion. In healthcare, we see this as a frequent feature of chronic inflammatory diseases, immunological diseases, cancer and neurological disorders. There are social (work, finances, etc) and genetic / molecular mechanisms that may aggravate fatigue. Certainly sickness behavior as it is called is seen in animals when infected in danger situations. The animal will exhibit sleepiness, depressive mood, social withdrawal, stop grooming, loose appetite and is an automated behavior thought to help protect the sick animal. People also exhibit these traits and can be due to chronic illness. Oxidative stress occurs with chronic inflammation. Heat shock proteins (HSPs) are proteins that protect under these conditions. These proteins can be intra and extracellular. They may play a role in signaling in chronic fatigue.

For pSS, there is no effective drug treatment. Could HSPs help us understand these two things, pSS and fatigue? pSS is associated with ocular / oral / and salivary gland symptoms. There may be focal sialoadenitis and anti-Ro/La antibodies (considered to be the most specific markers for pSS). Ocular dye tests (fluorescein stain for corneal surface and lissamine green for conjunctival surface) as well as Schirmer’s tear test can be done. Parotid sialography (to assess for diffuse sialectasis according to the scoring system of Rubin and Holt) as well as salivary scintigraphy according to the method proposed by Shall et al can be done. Hepatitis C must be excluded to diagnose pSS. Hepatitis C (HCV) may mimic the clinical, histological, and immunological features of pSS. Because HCV related sicca syndrome can meet criteria for pSS diagnosis, it must be excluded. Secondary Sjogren’s syndrome may be found with anticholingeric agents, antidepressants, antihypertensives, parasympatholytic drugs, neuroleptic agents, and past head and neck radiation treatment.

So in conclusion, fatigue is a natural phenomenon the body utilizes to help protect. It should be evaluated and if a disease process such as pSS is found, possible help can be given.

I hope you found this thought stimulating and helpful. ENT does not treat chronic fatigue of course as a specialty, but we do treat disorders of the head and neck, essentially what happens above your clavicle. If you have questions or concerns, we want to help. You can reach us at MidMichigan Health as noted below.

Philip Harris MD FARS

OtoRhinoLaryngology-Head and Neck Surgery
General ENT, Cancer, Thyroid, Parathyroid, Sinus
989-495-3166 (cell)


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