Using the ESSDAI alone to define systemic Sjogren’s is problematic because:

1. The ESSDAI does not include “non-ESSDAI” EGM or general systemic features. Patients with non-ESSDAI systemic manifestations may be incorrectly considered to have sicca-only disease because their ESSDAI score is zero.

2. The ESSDAI is a time-consuming, detailed research tool. Therefore, patients are rarely evaluated for an ESSDAI score, or if they are, the evaluation is often incomplete. 

3. Researchers sometimes perform a review of medical records to determine ESSDAI scores. This retrospective approach leads to artificially lower ESSDAI scores because of incomplete assessment and documentation.

4. The ESSDAI measures disease activity, not disease damage. It does not reflect past systemic involvement or even current stable symptoms caused by past disease damage. For example, a person could rate as “0/ no activity” in the pulmonary domain because of stable respiratory symptoms caused by old damage. Complicating matters further, it can be difficult to tell if long lasting features are stable, especially when no previous tests are available. (150)

5. Several ESSDAI domains tend be to be greatly underestimated, resulting in untreated systemic disease and unchecked disease progression.
    

​Commonly overlooked ESSDAI features:

Pulmonary (lung) disease is now understood to impact most Sjogren’s patients, including many without obvious symptoms. While most studies estimate a lung disease prevalence of 9-24%, these percentages are based on studies of symptomatic patients. CT scan abnormalities are found in 30-65% of patients who have no or mild symptoms (54,102).  Many patients who report shortness of breath and/or chronic cough are told that they are “out of shape” and that their cough is due to sicca. They deserve an evaluation; lung disease is often far progressed by the time it is diagnosed.

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Moderate, or even severe, interstitial lung disease (ILD) may be present in asymptomatic patients (89).  In a series of 51unselected Sjogren's patients, 41% overall (and 34% of never-smokers) met the GOLD criteria for chronic obstructive pulmonary disease (COPD) (158). ILD and COPD are not the only types of Sjogren's lung disease. These findings have led some experts to question the wisdom of only screening patients with symptoms because early identification and treatment improves outcomes (54, 89, 150). 

Note: "unselected" means patients were studied regardless of symptoms, history, physical exam findings, or other characteristics that would suggest lung disease or risk for lung disease.

See this blog post about the Clinical Practice Guidelines for pulmonary disease, and how you can use them to advocate for screening, with or without symptoms.

Small Fiber Neuropathies (SFNs)

“Painful small fiber neuropathy is the most common type of neuropathy in autoimmune patients, including SS (Sjogren’s).”  ~ Vivino et al, (151, p.155) 

Painful SFNs typically cause burning, tingling, and numbness in the feet and legs, although other areas of the body can be affected. SFNs are often overlooked (87, 96, 138) because they are undetectable by the typical neurologic examination and by electrodiagnostic (EMG/ nerve conduction velocity) tests. Formal ESSDAI assessments are often limited to large fiber and motor neuropathies, which are verifiable by neurologic examination and electrodiagnostic tests.

Dysautonomias impact up to 50% of Sjogren’s patients (50, 53), yet dysautonomia is not mentioned by the ESSDAI. In many patients, dysautonomia symptoms (cardiovascular, GI, bladder control issues, abnormal sweating and others) are inappropriately attributed to anxiety or functional neurologic disorder (32, 33).