Primary Sjögren's syndrome: central and peripheral nervous system involvements

Area of Science:

• Rheumatology
• Neurology
• Immunology

Background:

• Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease primarily causing exocrinopathy (dry eyes/mouth), fatigue, and pain.
• Neurological involvement in pSS is less common, affecting the central nervous system (2-5%) and peripheral nervous system (5-15%).

Purpose of the Study:

• To outline the diagnostic considerations for pSS, focusing on key symptoms and objective measures.
• To emphasize the importance of excluding differential diagnoses in patients with neurological symptoms.
• To describe the factors influencing treatment decisions for neurological manifestations in pSS.

Main Methods:

• Review of diagnostic criteria for pSS, including ACR/EULAR guidelines.
• Discussion of differential diagnoses for neurological symptoms in pSS patients.
• Analysis of treatment parameters for neurological involvement in pSS.

Main Results:

• pSS diagnosis is supported by symptoms like dry eyes/mouth, fatigue, pain, and objective measures.
• Neurological symptoms necessitate exclusion of conditions like cryoglobulinaemic vasculitis and multiple sclerosis.
• Treatment for pSS neurological issues considers cryoglobulinaemic vasculitis, symptom severity, progression, and prior treatment outcomes.

Conclusions:

• Accurate diagnosis of pSS relies on characteristic symptoms and objective findings.
• Distinguishing pSS-related neurological issues from other conditions is crucial for appropriate management.
• Tailored treatment strategies are essential for addressing neurological complications in pSS.