Sjogren Syndrome

Introduction

Sjogren’s Syndrome is an autoimmune disease that targets the lacrimal or crying glands and salivary duct in our mouth. This causes a constellation of symptoms that I remember with the GEM acronym. This includes gland enlargement, dry eyes and dry mouth. However it can also trigger dangerous extra glandular issues like lymphoma.
Now to classify this disease, think of it as either primary, where it occurs alone or secondary if it overlaps with rheumatoid arthritis or autoimmune diseases.

Further investigation for Sjogrens syndrome is required if a patient has significant sicca symptoms. A diagnosis requires both evidence of an underlying autoimmunity and objective glandular changes.

Autoimmunity can be shown in three ways. Firstly, a positive serology for
1. anti-Ro or anti-La. Secondly, a
2. biopsy of the minor salivary glands which show lymphocytic infiltrates. Finally, if a patient has a well-established systemic
3. rheumatic disease.

Evidence of glandular damage can be shown in four ways. Firstly, a
1. Schirmer test which measures tear production by placing a strip of paper over the lower eyelid and quantifying how wet it gets.
2. Ocular surface staining can be done to empirically measure eye damage caused by dryness.
3. Whole Sialometry is a method to quantify salivary hypofunction. Finally,
4. Imaging like an MRI or US can show evidence of parenchymal abnormalities.

Let’s expand on its clinical features by breaking down the GEM issues. Don’t forget to subscribe if you’re loving my channel’s mnemonics.

G stands for enlarged glands due to chronic inflammation. These are usually
- firm and nontender with either
- unilateral or bilateral enlargement. This is most obvious in the
- Parotid glands.
E stands for eyes that are dry and irritated due to a reduction in the
- aqueous component of tears. This is medically termed
- keratoconjunctivitis sicca and gives patients an unpleasant
- “Gritty” or “sandy” feeling in their eyes.
M stands for dry mouth caused by
- Chronic salivary hypofunction. This is medically described as
- “xerostomia”. Decreased saliva production increases the risk of
- Dental caries and
- oral candidiasis

If you have gems, you might also have a MAP which is my acronym for the extra glandular features of SS.

M stands for malignancy, specifically lymphoma. This is the most serious complications of SS and confers up to a
- ninefold increase in risk. The tumours are usually
- extranodal arising within exocrine glands.
A stands for Antibody Deposition.
- Immune complex–mediated disease often causes a strange
- Vasculitis or dermatological stigmata like a
- Rash. Finally,
P stands for Periepithelitis. This describes the immune response in
- ductal epithelial structures within a
- Variety of organs like the kidney, causing things like
- interstitial nephritis

Don’t forget that other non-specific symptoms like Raynaud Phenomenon and fatigue are common too.

Let’s recap with my mnemonic that summarises its diagnostic criteria. SS requires proof of autoimmunity like, a positive anti-Ro or anti-La, and evidence of glandular dysfunction which you can quantify with Dye, Cry, MRI, or Saliva.

The dye is a reminder that you can do ocular surface staining, reduced crying can be measured with a Schirmer test, tissue damage can sometimes be seen on imaging, and saliva hypofunction can be measured with Sialometry.