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Updated: Jun 24, 2026
07:25
Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights
Published on: October 13, 2023
[Vasculopathy in Sjögren's syndrome]
E Feist1, K-G A Hermann, A Dankof
1Klinik für Rheumatologie und Klinische Immunologie, Charité-Universitätsmedizin Berlin, Charitéplatz 1, Berlin, Germany. eugen.feist@charite.de
Zeitschrift Fur Rheumatologie
|April 10, 2009
Summary
Sjögren's syndrome can cause systemic vasculitis in 5-10% of patients, often linked to B-cell activity. Treatment involves immunosuppressants, with Rituximab considered for severe cases.
Area of Science:
- Rheumatology
- Immunology
- Pathology
Context:
- Sjögren's syndrome is a systemic autoimmune disorder primarily affecting exocrine glands, causing sicca symptoms.
- Extraglandular involvement impacts skin, musculoskeletal, neurological systems, and organs in approximately 40% of patients.
- Systemic vasculitis occurs in 5-10% of Sjögren's syndrome patients, manifesting as leukocytoclastic or cryoglobulinemic types.
Purpose:
- To review the pathogenesis, clinical manifestations, prognostic factors, and treatment strategies for vasculitis in Sjögren's syndrome.
- To highlight the role of B-cell activation and autoantibodies in the development of vasculitis.
- To identify negative prognostic markers and discuss therapeutic options for Sjögren's-associated vasculitis.
Summary:
- Vasculitis in Sjögren's syndrome is associated with B-cell hyperactivity, leading to autoantibodies (Ro/SS-A, La/SS-B) and cryoglobulins.
- Negative prognostic indicators include vasculitis, non-Hodgkin's lymphoma, glomerulonephritis, positive cryoglobulins, and low complement levels.
- Standard treatment combines immunosuppressants with glucocorticoids; Rituximab is an option for refractory or severe cases.
Impact:
- Understanding vasculitis pathogenesis in Sjögren's syndrome aids in identifying high-risk patients.
- Recognition of negative prognostic markers allows for timely and aggressive therapeutic interventions.
- B-cell-targeted therapies like Rituximab offer new hope for managing severe Sjögren's syndrome-associated vasculitis.
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