Sjögren's syndrome with and without neurological involvement

Area of Science:

• Rheumatology
• Neurology
• Clinical Immunology

Background:

• Neurological manifestations in primary Sjögren's syndrome (pSS) can be severe yet treatable.
• Identifying patients with pSS and neurological involvement (pSSN) is crucial for timely diagnosis and management.

Purpose of the Study:

• To systematically evaluate the neurological manifestations in primary Sjögren's syndrome.
• To identify clinical features that help distinguish pSSN patients from those with pSS without neurological involvement.

Main Methods:

• A cross-sectional study compared para-/clinical features of 512 patients diagnosed with pSS/pSSN using 2016 ACR/EULAR criteria.
• Patients with suggestive neurological symptoms were screened for Sjögren's syndrome.
• Disease activity in pSSN was assessed using the Neurological Involvement of Sjögren's Syndrome Disease Activity Score (NISSDAI).

Main Results:

• 46% of the cohort (238/512) had pSSN.
• Independent predictors for neurological involvement included male sex, older age at onset, hospitalization, lower IgG, and higher eosinophil counts.
• Additional univariate predictors in pSSN were older age at diagnosis, lower rheumatoid factor, presence of SSA(Ro)/SSB(La) antibodies, higher white blood cell count, and CK levels.

Conclusions:

• Patients with pSSN exhibit distinct clinical characteristics compared to pSS patients.
• Neurological involvement in Sjögren's syndrome appears to be underestimated.
• Intensified neurological screening is recommended for the diagnostic algorithm of Sjögren's syndrome, particularly for older males with a severe disease course requiring hospitalization.