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Sjogren's Sydrome (Physiology of Disease (A systemic autoimmune…
Sjogren's Sydrome
Physiology of Disease
A systemic autoimmune disease characterized by lymphocytic infiltrations of lacrimal and salivary glands.
Thought to be multi-step; triggered by an environmental factor (likely viral) in a genetically pre-disposed individual.
Beginning events trigger innate immune system but due to prolonged propagation and perpetuation is in constant interplay between innate and adaptive.
The result is autoreactive B-cell stimulation, autoantibody production, and chronic inflammation of the salivary and lacrimal glands and often other tissues.
Chronic stimulation of B cells in the target tissue may promote lymphomagenesis, again through a multistep process in a genetically susceptible individual.
First body attacks the glands that make tears and saliva, but can also damage other areas such as the joints, thyroid, kidneys, liver, lungs, skin, and nerves.
Risk factors include age (as SS is usually diagnosed in individuals over 40), sex (women are more likely to develop SS), and Rheumatic disease such as rheumatoid arthritis or lupus (common to see the two paired).
Alterations in Function & Diagnosis Criteria
Marked increased risk of developing lymphoma in comparison to other autoimmune disorders
SS shows altered levels of specific types of immune T-cells and B-cells, which may play an important role in disease activity.
Blood Tests
Should check blood levels for the different blood cells, the presence or absence antibodies, evidence of inflammation, liver and/or kidney malfunction indicators.
Eye Tests
Schirmer tear test- filter paper is placed under the lower eyelid to detect the production of tears.
Examine surface of eye using a slit lamp. Damage to cornea may be present in which an ophthalmologist will place drops in the eye.
Imaging
To check the functions of the salivary glands
Sialogram- Dye is injected in salivary glands in which the X-ray technology will detect production of saliva and its flow.
Salivary scintigraphy- Radioactive isotope is injected into a vein which is then tracked to see how long it takes to approach the salivary glands.
Biopsy
While not needed, a doctor can test a piece of an individual's lip for inflammatory cells.
Clinical Manifestations
General Manifestations
Dry eyes
Dry mouth
Dry skin and rashes
Fatigue
Joint pain and stiffness
Dry cough
Systemic Extraglandular Manifestations
Arthritis
Nephritis
Cytopenia
Pneumonitis
Vasculitis
Lupus
Neurologic Manifestations
Peripheral neuropathy
Myelopathy
Cognitive disturbances
Treatment Options
Medications
Prescription eyedrops (Restasis) to decrease eye inflammation.
Salagen or Evoxac to increase production of saliva and sometimes tears. Some side effects are sweating, abdominal pain, flushing, and increased urination.
NSAIDs to accomodate possible clinical manifestations such as arthritis.
Antifungal medications to reduce yeast infections in the mouth.
Drugs that suppress the immune system may also be used in severe cases.
Surgery
A minor surgery involving the closing of tear ducts that drain tears from the eyes which could help dry eyes. The ducts are then plugged with collagen or silicone to preserve tears.
Lifestyle Changes
Relief of dry eyes
Use of artificial tears or eye lubricants.
Increasing indoor humidity can also help.
Relief of dry mouth
Cease smoking.
Increase fluid intake.
Sugarless gum or hard candies can increase saliva flow to mouth.
Artificial saliva.
Nasal spray to moisturize and clear nasal cavity which limits mouth breathing.
Dry mouth can cause a multitude of issues so regular oral health behaviors are encouraged such as brushing teeth and flossing after every meal, regular dental appointments, and using fluoride treatments/antimicrobial mouthwashes.