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Hyperviscosity in primary Sjögren's syndrome: clinical implications.
Gabriela Hernández-Molina1, Paola Bermúdez-Bermejo1
1Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición SZ, Mexico City, Mexico.
International Journal of Rheumatic Diseases
|January 26, 2016
Summary
High serum viscosity affects nearly half of primary Sjögren's syndrome patients, often linked to vasculitis. Classic hyperviscosity syndrome is rare but also associated with vasculitis and splenomegaly, suggesting distinct clinical implications.
Area of Science:
- Rheumatology
- Immunology
- Clinical Medicine
Background:
- Primary Sjögren's syndrome (pSS) is associated with increased serum viscosity.
- Classic hyperviscosity syndrome (HVS) is a rare complication in pSS.
- Understanding the clinical and serological differences between pSS with and without HVS is crucial.
Purpose of the Study:
- To compare clinical and serological profiles of pSS patients with HVS, high viscosity without HVS, and normal viscosity.
- To identify factors associated with HVS in pSS.
- To elucidate the distinct implications of high viscosity and HVS in pSS.
Main Methods:
- Retrospective analysis of four pSS patients with HVS.
- Inclusion of 62 pSS patients as controls.
- Measurement of serum viscosity, C3, C4, immunoglobulins, and ESSDAI scores.
- Statistical analysis including chi-squared, Mann-Whitney U tests, and logistic regression.
Main Results:
- High viscosity (≥1.9 cP) was found in 48.3% of pSS patients.
- HVS cases presented with higher IgG, IgM, IgA, vasculitis, neutropenia, lymphopenia, and splenomegaly.
- Vasculitis (OR 14.8) and splenomegaly (OR 25.3) were significantly associated with HVS.
- High viscosity without HVS correlated with higher ESSDAI scores and more vasculitis.
Conclusions:
- High serum viscosity is common in pSS and linked to vasculitis and disease activity.
- HVS is infrequent in pSS but associated with vasculitis and splenomegaly.
- High viscosity and HVS appear to have different pathophysiological and clinical implications in pSS.
Keywords:
Sjögren‘s syndromehyperviscosityhyperviscosity syndromeserological activityvasculitisviscosityMore Related Videos
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