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BLyS upregulation in Sjogren's syndrome associated with lymphoproliferative disorders, higher ESSDAI score and B-cell clonal expansion in the salivary glands.
- Source :
-
Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2013 Feb; Vol. 52 (2), pp. 276-81. Date of Electronic Publication: 2012 Aug 09. - Publication Year :
- 2013
-
Abstract
- Objective: Primary SS is characterized by an increased risk of lymphoma in patients with prelymphomatous manifestations (i.e. myoepithelial sialadenitis or mixed cryoglobulinaemia). Serum B-lymphocyte stimulator (s-BLyS) levels in SS-related B-cell lymphoproliferative disorders were studied by integrating the results with the disease activity score and with molecular analyses of B-cell expansion in the salivary glands.<br />Methods: Seventy-six primary SS patients (with or without lymphoma or prelymphomatous manifestations), 56 HCV-related cryoglobulinaemic vasculitis patients and 55 controls were studied. s-BLyS and molecular analyses of B-cell expansion in the salivary gland tissues were performed. Patients with SS and persistent parotid swelling underwent parotid biopsy.<br />Results: s-BLyS differed between SS subgroups, higher levels being documented in patients with lymphoma or prelymphomatous manifestations vs SS without [1.85 (0.45-4.12) ng/ml vs 1.12 (0.56-1.98) ng/ml; P < 0.0001]. s-BLyS levels significantly correlated with the European League Against Rheumatism (EULAR) SS disease activity index (r = 0.62, P < 0.0001, Spearman's test). Clonal B-cell expansion in the salivary glands, but not polyclonal B-cell expansion, was associated with higher s-BLyS levels [1.98 (0.45-4.12) ng/ml vs 1.15 (0.56-3.25) ng/ml; P = 0.013)].<br />Conclusion: Higher s-BLyS levels and tissue clonal B-cell expansion characterize SS with B-cell lymphoproliferative disorders, even at prelymphomatous stages. This subgroup of SS patients showed the highest EULAR SS disease activity index scores. This represents a biologic rationale for targeting both clonal B-cell expansion and s-BLyS overproduction in SS.
- Subjects :
- Adult
Aged
Biopsy
Case-Control Studies
Cell Proliferation
Clone Cells
Enzyme-Linked Immunosorbent Assay
Female
Humans
Male
Middle Aged
Polymerase Chain Reaction
Severity of Illness Index
B-Cell Activating Factor blood
B-Lymphocytes immunology
Lymphoproliferative Disorders blood
Salivary Glands immunology
Sjogren's Syndrome blood
Up-Regulation physiology
Subjects
Details
- Language :
- English
- ISSN :
- 1462-0332
- Volume :
- 52
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Rheumatology (Oxford, England)
- Publication Type :
- Academic Journal
- Accession number :
- 22879463
- Full Text :
- https://doi.org/10.1093/rheumatology/kes180