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Hyperviscosity in primary Sjögren's syndrome: clinical implications
Author(s) -
HernándezMolina Gabriela,
BermúdezBermejo Paola
Publication year - 2017
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12751
Subject(s) - medicine , rheumatoid factor , hyperviscosity , gastroenterology , serology , vasculitis , rheumatism , hyperviscosity syndrome , odds ratio , blood viscosity , immunology , rheumatoid arthritis , antibody , disease , multiple myeloma
Abstract Aim Increased serum viscosity is recognized in primary Sjögren's syndrome ( pSS ); however, a classic hyperviscosity syndrome ( HVS ) is rare. We compared the clinical and serological profile among three groups of pSS patients: (i) with HVS ; (ii) with high serum viscosity (≥ 1.9 cP [centipoises]) but without HVS ; and (iii) with normal viscosity (< 1.9 cP). Methods We identified four pSS patients with HVS and retrospectively assessed their clinical/serological features. We included as controls 62 pSS patients and registered their clinical features. We also measured the serum viscosity, C3, C4, immunoglobulins and evaluated the European League Against Rheumatism SS Disease Activity Index ( ESSDAI ) score at the last visit. We used χ 2 , Mann–Whitney U ‐tests and logistic regression analysis. Results Patients were predominantly female (95%), mean age 54 ± 14.2 years, median disease duration 9 years. All the HVS cases were diagnosed concomitantly with the onset of SS and had higher titers of immunoglobulin G (IgG), IgM, IgA and a higher prevalence of vasculitis, neutropenia, lymphopenia and splenomegaly. At the multivariate analysis, the variables vasculitis odds ratio ( OR ) 14.8 (95% CI 1.3–99, P = 0.02) and splenomegaly OR 25.3 (95% CI 1.68–380, P = 0.01) remained associated with HSV . Viscosity levels correlated with rheumatoid factor titers. Thirty (48.3%) patients had high viscosity but without HSV ; this group had higher median ESSDAI scores and more vasculitis than patients with normal viscosity. Conclusion High viscosity was present in almost half of the patients and was associated with vasculitis and higher activity scores. Conversely, HVS was infrequent and was associated with vasculitis and splenomegaly. It seems that both conditions have different physiopathological, clinical and treatment implications.