Sjogren syndrome: Clinical practice

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Sjogren syndrome: Clinical practice

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A 26-year-old woman comes to the office because of a 1-week history of foreign body sensation in both eyes and eye redness. Her medical history is relevant to a recent and uncomplicated laser refractive eye surgery. The patient complains that for the past few days, she wakes up in the morning with a whitish mucoid discharge in both eyes and a blurry vision. Eye exam shows corneal xerosis and conjunctival redness. Her temperature is 36.7°C (98.2°F), pulse is 61/min, respirations are 16/min, blood pressure is 110/60 mmHg. Which of the following is the most likely diagnosis? 

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Content Reviewers:

Rishi Desai, MD, MPH

Sjogren's syndrome is a common autoimmune disorder, that mostly occurs in females, where the immune system attacks various exocrine glands, which are glands that pour their secretions into a duct.

Most commonly the salivary glands and the lacrimal or tear glands are affected.

If Sjogren syndrome is primary or occurs alone - it’s called sicca syndrome, and that’s associated with anti-SSA/RO and anti-SSB/LA antibodies.

Alternatively it can be secondary, which means that it is accompanied by other autoimmune diseases like lupus, rheumatoid arthritis, and scleroderma.

Sjögren's syndrome causes dryness of various body surfaces.

Lacrimal gland involvement leads to dryness of the eyes, blurry vision, itching, redness and burning and ultimately to keratoconjunctivitis, which is the inflammation and ulceration of the cornea and conjunctiva.

Salivary gland involvement leads to xerostomia, or dry mouth, difficulty in tasting and swallowing, cracks and fissures in the mouth, and eventually tooth decay.

In the nose and airways, it causes ulceration and bleeding, and if this affects the larynx, it can lead to difficulty speaking.

In some people there may be dryness of the skin and vagina.

Finally the salivary and lacrimal glands can swell up and compress nearby structures like nerves, causing pain.

In addition, Sjögren's syndrome can also affect organs beyond the exocrine glands as well, and sometimes it can overlap into another autoimmune disorder.

Systemic symptoms include fever, fatigue, myalgia, unintentional weight loss, and lymphadenopathy.

There can be vascular conditions like palpable purpura, due to bleeding within the skin.

These purpura can develop into large ulcers that can get infected.

Another vascular condition is Raynaud’s phenomenon, which is where arterial spasm reduce the blood flow to the fingers for a few minutes at a time.

The fingers turn white and then blue, often with numbness or pain, and then as blood flow returns, the fingers turn red and burn.

Lung problems include a chronic cough, as well as interstitial lung disease.

Renal involvement can cause interstitial nephritis and defects in tubular function, causing creatinine levels to rise.

More rarely, it can cause glomerulonephritis, leading to hematuria and proteinuria.

Gastrointestinal problems include dysphagia, esophageal motility disorders, dyspepsia, gastritis, celiac disease, liver abnormalities like primary biliary cirrhosis or autoimmune hepatitis, and pancreatic diseases like autoimmune sclerosing pancreatitis.

Hematologic manifestations in Sjogren’s include mild anemia and leukopenia; as well as hypergammaglobulinemia; monoclonal gammopathies, cryoglobulinemia, and lymphoma.

Finally, some individuals with Sjogren’s may develop autoimmune thyroiditis.

Since virtually any organ or gland can be affected, there is a wide variety of diagnostic tests that can be done.

Starting from the eyes, there are three commonly used tests for dry eye.

First, is the Schirmer test, which measures reflex tear production.

A folded test strip of sterile filter paper, supplied in a standard kit, is placed over the margin of each lower eyelid at the junction of the middle and lateral thirds. The extent of wetting is measured over five minutes: wetting of less than 5 millimeters suggests aqueous tear deficiency.

Second, is ocular surface staining. That’s where dyes like rose bengal, fluorescein, or lissamine green, are used to stain areas of damaged tissue. The ocular surface is then examined with a slit lamp to assess the damage to the conjunctiva and cornea.

Third, there’s the tear break-up time.

Now, when we blink, a film of tears spreads over the eye, making the surface of the eye smooth and clear. Without this tear film, good vision would not be possible.

The tear break-up time is how long it takes for a dry spot to appear within this tear film - disrupting it.

The tear break-up time is determined by staining the tear film with one drop of fluorescein dye and measuring the time in seconds for a dry spot to develop. The tear film is observed with a slit lamp and cobalt blue light, and a tear break-up time of less than 10 seconds indicates a problem in the outermost mucus layer of the tear film.

Next up, are tests that help identify salivary gland hypofunction.

First, there’s quantitative salivary gland scintigraphy, also called technetium excretion radionuclide scanning, which shows how the major salivary glands are functioning based on their uptake of a radionuclide.