[Central nervous system involvement in primary Sjögren syndrome]

Area of Science:

• Rheumatology
• Neurology
• Immunology

Context:

• Sjögren syndrome (SS) is a systemic autoimmune disorder primarily affecting exocrine glands, leading to sicca symptoms.
• While SS commonly causes sicca symptoms, it can also manifest as extraglandular involvement, including the central nervous system (CNS).

Purpose:

• To review the spectrum of central nervous system (CNS) involvement in primary Sjögren syndrome (SS).
• To highlight the decreased frequency of symptomatic CNS involvement in recent studies and discuss the differential diagnosis.

Summary:

• Symptomatic central nervous system (CNS) involvement in primary Sjögren syndrome (SS) is infrequent (<5%) in recent large series, contrasting with earlier findings.
• Neurological manifestations range from asymptomatic white matter lesions on MRI to severe focal or diffuse neurological deficits.
• Differential diagnosis is crucial, considering overlapping conditions like lupus, antiphospholipid syndrome, vasculitis, cerebrovascular disease, and multiple sclerosis.

Impact:

• This review underscores the importance of considering neurological complications in Sjögren syndrome (SS) patients.
• It emphasizes the need for a thorough differential diagnosis to accurately identify the cause of neurological symptoms in the context of SS.
• Understanding the varied neurological presentations aids in timely diagnosis and management of Sjögren syndrome (SS).