Neurological involvement in Primary Sjögren Syndrome

Area of Science:

• Neurology
• Rheumatology
• Immunology

Background:

• Primary Sjögren syndrome (pSS) is a systemic autoimmune disease primarily affecting exocrine glands.
• Neurological complications are recognized but their prevalence and spectrum in pSS require further elucidation.

Purpose of the Study:

• To evaluate the prevalence, clinical patterns, and outcomes of neurological involvement in a cohort of primary Sjögren syndrome patients.
• To compare clinical and seroimmunological data between pSS patients with and without neurological involvement.

Main Methods:

• Observational, retrospective, cross-sectional case-control study.
• Reviewed clinical data of 93 primary Sjögren syndrome patients diagnosed by 2002 American-European Consensus Group criteria.
• Compared demographic, clinical, and seroimmunological data between patients with and without neurological complaints.

Main Results:

• Neurological involvement was detected in 26% (28/93) of pSS patients, with symptoms preceding diagnosis in 46% of cases.
• Peripheral nervous system (PNS) involvement (46%) included sensory neuropathy and cranial nerve palsies; central nervous system (CNS) involvement (50%) included headache, myelopathy, seizures, and cognitive dysfunction.
• Patients with neurological involvement showed higher frequencies of constitutional and lung symptoms, and lower frequencies of articular symptoms (p<0.05).
• Good neurological outcome was observed in 77% of patients.

Conclusions:

• Primary Sjögren syndrome presents with diverse neurological complications, frequently as an initial manifestation.
• The high frequency of neurological symptoms, particularly CNS involvement, may lead to underdiagnosis or delayed diagnosis of pSS.
• Systematic screening for pSS is recommended in patients with myelopathy, axonal sensorimotor neuropathy, or cranial nerve involvement.