SYSTEMIC SJOGREN'S

CATEGORY 2 - OUT OF ESSDAI FEATURES

Many systemic features of Sjogren's are not included in the ESSDAI.  

Researchers measure the systemic features of Sjogren's using a standardized research tool called the the ESSDAI. Many systemic manifestations are not included in the ESSDAI, including "Out of "ESSDAI" features. "Out of ESSDAI" features tend to be ignored by researchers and clinicians, even though they are common and usually measurable. In the clinical setting, these direct Sjogren's manifestations may help point to a Sjogren's diagnosis, especially for patients with non-sicca presentations (63).


Excluding "Out of ESSDAI" manifestations from the overall clinical picture, which is ultimately the correct way to approach diagnosis, reinforces the narrow, outdated view of Sjogren's as primarily a sicca disease. These features present a broader picture of Sjogren's, one that includes multiple systemic features that are part of the disease spectrum. 

 

"Out of ESSDAI" systemic features- a partial list 

  1. Dysautonomias are neurologic manifestations caused by a malfunctioning autonomic nervous system. Even though dysautonomias impact the majority of Sjogren’s patients (33, 50, 53), the ESSDAI guidelines(37) do not include them. These neurologic manifestations are often overlooked; rheumatologists may not be trained to recognize them. Cardiovascular dysautonomias such as POTS are treatable, contributors to fatigue (33, 49, 50). 
     

  2. Carpal tunnel syndrome is a common peripheral nervous system (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 the  peripheral nerves and multiple organs (30, 46).
     

  5. Gastrointestinal (GI) manifestations are nearly ubiquitous (112). Difficulty swallowing, not just  from 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 of Sjogren's. (2, 13).
     

  6. Extraglandular eye manifestations such as scleritis, iritis, and uveitis occur in a subset of patients. These serious inflammatory complications are not caused by sicca (40).
     

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

  8. Pregnancy complications, including congenital fetal heart block and neonatal lupus may occur in SSA (anti-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. These are more common in SLE, but do occur in Sjogren's (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 3-24-21