sicca syndrome

Today is a most bizarre weather-related day.  It’s warm, like you don’t need a coat warm, and there’s a raging thunderstorm.  Did I mention it’s February in upstate New York?  In honor of this most bizarre day, I’d thought I’d write a few words on a somewhat bizarre and illusive autoimmune disease called Sjögren’s syndrome.

Henrich Sjögren gave Sjögren’s syndrome its name.  He was a Swedish physician who first described the disease in 1933.  Sjögren’s syndrome is a common autoimmune disease that primarily causes dryness.  But it’s a lot more complicated than that because Sjögren’s syndrome can involve almost any organ so can present with a myriad of symptoms.  The symptoms arise from infiltration of lymphocytes into glands and affected organs.  Simply put, Sjögren’s syndrome is on the differential diagnosis in any person who has a positive ANA presenting with unexplained symptoms.

10 Warning signs you may be suffering from Sjögren’s syndrome

The following are some of the common manifestations of Sjögren’s syndrome.  Believe me, there are A LOT more but these are some of the common ones.

1. Dry eyes
2. Dry mouth
3. Swollen cheek(s) i.e., parotid gland enlargement
4. Profound tiredness
5. Joint pain, sometimes with swelling
6. Swollen glands
7. Numbness, tingling, burning of the skin
8. Raynaud’s
9. Shortness of breath with minimal work
10. Having a child that suffered from congenital heartblock

Dry mouth symptoms

The following are some common symptoms of dry mouth.

1. Difficulty swallowing dry foods
2. Inability to talk continuously
3. Change in taste
4. Burning sensation
5. Large dentist bill! – Cavities, cracked teeth, loose fillings
6. Problems with your dentures
7. Worsening heartburn
8. Thrush

As you can see the symptoms are a little all over the place and quite frankly are kind of vague.  Furthermore, many different conditions can mimic some of these symptoms: dehydration, depression, various medications, uncontrolled diabetes, multiple sclerosis, hepatitis C, sarcoidosis, etc etc.  Literally.

Classification criteria

Now it’s important to note that the following classification criteria are used for research purposes, and not necessarily for the day-to-day clinic.  Although they are important, there is such a thing called the art of medicine.

As we all know, not everyone fits into a neat little box.

Recently the American College of Rheumatology and the European League Against Rheumatism came up with a new system to classify Sjögren’s.  Basically, a group of hot-shot Sjögren’s specialists got together, looked at the literature, probably had more than one heated discussion, and came up with the following.

To test positive you need to have a score ≥4.  There are five items but they are weighted differently.

• 3 Points – Anti-SSA/Ro antibody positivity
• 3 Points – Focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm²
• 1 Point – Abnormal Ocular Staining Score of ≥5 (or van Bijsterveld score of ≥4)
• 1 Point – Schirmer’s test result of ≤5 mm/5 mi
• 1 Point – Unstimulated salivary flow rate of ≤0.1 mL/min, each scoring = 1

The sensitivity of this score is 96% and the specificity is 95%.  The sensitivity tells you how likely you are to detect all cases of Sjögren’s syndrome and the specificity tells you how accurate you are with the diagnosis using these set of diagnostic criteria.  These are pretty good figures.

What does this mean?

As you can see, the diagnosis favors objective findings, NOT symptoms.  This is a huge change from the previous set of diagnostic criteria.  You’ll also note that positive ANA, rheumatoid factor, and positive anti-SSB/La antibody positivity are not included in the new classification criteria.

Now I don’t want people thinking that I think symptoms are unimportant.  They are VERY important.  It’s just that symptoms should prompt a workup looking for objective features of the disease.

Now, try to remember the 10 warning signs.  If you find yourself checking a few of these items, check-in to your local rheumatologist.

References

Rheumatology Secrets 3rd edition

Shiboski CH, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts.Ann Rheum Dis. 2017 Jan;76(1):9-16.