Primary Sjogren's syndrome with central nervous system involvement

Area of Science:

• Neurology
• Immunology
• Rheumatology

Background:

• Primary Sjogren's syndrome (PSS) is an autoimmune disease primarily affecting exocrine glands.
• Central nervous system (CNS) involvement in PSS is a recognized but less common manifestation.
• Understanding the clinical spectrum of CNS involvement in PSS is crucial for diagnosis and management.

Purpose of the Study:

• To delineate the clinical, laboratory, and radiological characteristics of PSS patients with CNS involvement.
• To highlight the variability and potential early presentation of neurological symptoms in PSS.

Main Methods:

• Retrospective case series of 12 female patients diagnosed with PSS and CNS involvement.
• Data collected from 1991-2009 at King Faisal Specialist Hospital and Research Center.
• Analysis of clinical, radiological (MRI), and immunological features based on American-European Diagnostic Criteria.

Main Results:

• The mean age of patients was 40 years, all female, presenting with active neurological symptoms.
• Neurological symptoms preceded sicca symptoms in 33% of cases; myelopathy (66%) and optic neuritis (75%) were common.
• MRI revealed white matter changes in the brain (58%) and spinal cord (50-16%).

Conclusions:

• CNS involvement in PSS exhibits significant clinical variability and can manifest years before sicca symptoms.
• PSS with CNS involvement can mimic multiple sclerosis, necessitating consideration of PSS screening.
• Further research with larger cohorts is needed to establish definitive management protocols.