Systemic Sjogren's Category 2:

Out of ESSDAI features

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 There are numerous distinct systemic Sjogren's features not included in the ESSDAI. The following is a partial list of these specific (as opposed to general) features. To distinguish the two categories these are labeled ) "out of ESSDAI" systemic features.

Even though these are an important part of the spectrum of disease, they often remain overlooked in the clinic setting. They are rarely taken into consideration when making a Sjogren's diagnosis (63). Excluding them from the overall clinical picture, which is ultimately the correct way to approach diagnosis, reinforces the outdated view that Sjogren's is primarily a sicca disese.

 

Sjogren's Classification Criteria are heavily weighted toward sicca manifestations, reinforcing the tendency to ignore the multitude of systemic features that are part of the disease spectrum. Classification should never replace the clinician's expert opinion in making the diagosis of Sjogren's. 

"Out of ESSDAI" systemic features- a partial list 

  1. Dysautonomias are neurologic manifestations that impact the majority of Sjogren’s patients (33, 50, 53). The ESSDAI guidelines (37) do not list them as a PNS feature, although it would be logical to include them in that category. These are often overlooked; rheumatologists may not be trained to recognize them. Cardiovascular dysautonomias such as POTS are a treatable contributor to fatigue (33, 49, 50). 
     

  2. Carpal tunnel syndrome is another common PNS complication that is not included in the ESSDAI scoring system.  
     

  3. Raynaud's phenomenon: a systemic autoimmune feature affecting 10-30% of individuals with Sjogren’s. (3, 81
     

  4. Vasculitis of organs in addition to the skin and CNS. Thought to be uncommon but may affect peripheral nerves and multiple organs. (30, 46).
     

  5. Gastrointestinal (GI) manifestations are nearly ubiquitous. (112) Difficulty swallowing, not just due to sicca (148), GERD, and chronic gastritis, gut dysmotility including gastroparesis, and SIBO/dysbiosis frequently occur. (113) Autoimmune hepatitis, primary biliary cholangitis and pancreatitis are uncommon but serious organ system complications (2, 13).
     

  6. Extraglandular eye manifestations such as scleritis, iritis, and uveitis are uncommon but serious inflammatory complications of the eye.  Although these conditions impact the eyes, they are not related to sicca (40).
     

  7. Severe headaches, including migraines, are as about as common in Sjogren’s as they are in systemic lupus erythematosus (SLE). (41)
     

  8. Pregnancy complications, including congenital fetal heart block and neonatal lupus may occur in SS-A (Ro) positive women. (75) Patients with antiphospholipid antibodies are at high risk of blood clots and miscarriage.
     

  9. Serositis, including pericarditis, pleuritis and abdominal serositis.  More common in SLE, but do occur. (63
     

  10. Interstitial cystitis, overactive bladder and frequent UTI. (63)
     

  11. Sensorineural hearing loss (73)
     

  12. Restless leg syndrome (63

 From the review article about systemic manifestations of primary Sjogren's syndrome out of the ESSDAI classification:

" The results of this study, together with the already-published evidence supporting a pivotal role of systemic disease in primary SS, are pointing out the need of a future re-evaluation of how we are defining, classifying and diagnosing primary Sjogren's." 

~ S Retamozo et al, 2019 (63

Updated 8-2-20