SYSTEMIC SJOGREN'S

CATEGORY 2 - OUT OF ESSDAI FEATURES

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

Most researchers study only the systemic features associated with the ESSDAI. “Out of ESSDAI” features tend to be downplayed or ignored in both clinic and research settings. When "Out of ESSDAI" manifestations (63) are included in the assessment, they can help formulate a Sjogren’s diagnosis, especially for patients who do not fit the classification criteria, such as the 20% with non-sicca presentations.

 

Excluding "Out of ESSDAI" manifestations from the overall clinical picture, which is ultimately the correct way to approach diagnosis, reinforces misperceptions about the disease. Dysautonomia and small fiber neuropathy are the most common neurological features of Sjogren’s. They frequently precede sicca and are more likely to occur in SS-A negative patients. Despite their major impact on quality of life, symptoms are often overlooked, dismissed, or psychologized.

 

"Out of ESSDAI" systemic features- a partial list 

  1. Autonomic disorders (Dysautonomia) are neurologic manifestations caused by a malfunctioning autonomic nervous system. Even though surveys show that autonomic dysfunction, even when mild, impacts the majority of Sjogren’s patients (33, 50, 53), the ESSDAI guidelines(37) do not include them. Many rheumatologists are not trained to recognize them. Cardiovascular versions of dysautonomia such as postural orthostatic tachycardia syndrome (POTS) are treatable contributors to fatigue (33, 49, 50). 
     

  2. Small fiber neuropathy is theoretically part of the ESSDAI, (PNS). However, clinicians and researchers may not be familiar with the diagnosis. 
     

  3. Carpal tunnel syndrome is a common peripheral nervous system (PNS) complication.  
     

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

  5. 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, 207).
     

  6. 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). 
     

  7. Extraglandular eye manifestations such as scleritis, iritis, and uveitis occur in a subset of patients.  Althoug these conditions occur in the eye, they are not related to sicca(40).
     

  8. Severe headaches, including migraines, are as about as common in Sjogren’s as they are in systemic lupus erythematosus (SLE) (41). Headache is the most common neurological feature of SLE, and understood to be directly caused by the disease. This has been poorly studied in Sjogren's, despite evidence suggesting that this is a common Sjogren's feature (206). 
     

  9. 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.
     

  10. Serositis, including pericarditis, pleuritis, and abdominal serositis. These are more common in SLE, but do occur in Sjogren's (63). 
     

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

  12. Sensorineural hearing loss (73)
     

  13. Restless leg syndrome (63) and other sleep disorders. 

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 08-16-2022