Objectives. Serum immunoglobulin free light chains (FLCs) are frequently elevated in B-cell-mediated autoimmune diseases, including primary SS (pSS). The objective of this study was to assess if serum FLCs can contribute to classification, mucosa-associated lymphoid tissue (MALT) lymphoma detection, monitoring of disease activity and treatment response in pSS.
Methods. Serum samples of 100 consecutive patients suspected of pSS were included. Forty-five patients fulfilled ACR-EULAR criteria for pSS. Additionally, samples of 17 pSS patients with MALT lymphoma and longitudinal samples of pSS patients treated with rituximab (n = 20), placebo (n = 10) or abatacept (n = 15) were included. Serum FLC kappa/FLC lambda. was measured by nephelometry or turbidimetry.
Results. At diagnosis, FLC kappa and FLC lambda serum levels were significantly higher in pSS compared with non-SS sicca patients. The FLC kappa/FLC lambda ratio was abnormal in 11% of pSS patients. In established MALT-pSS patients, without recent rituximab treatment (n = 12), 50% had abnormal FLC kappa/FLC lambda ratios. FLC measurement had no additional value for pSS classification, compared with IgG and anti-SSA. FLC levels correlated significantly with systemic disease activity, assessed by EULAR SS Disease Activity Index (ESSDAI) and clinical ESSDAI, both cross-sectionally and longitudinally following treatment. Treatment with rituximab or abatacept significantly lowered FLC levels. FLCs show a large sensitivity to change and relative changes induced by treatment were higher compared with IgG.
Conclusion. Serum FLCs are elevated in pSS, and abnormal FLC kappa/FLC lambda. ratios may be indicative for the presence of MALT lymhoma. FLC levels can be used as a biomarker for systemic disease activity and monitoring treatment responses. FLCs are sensitive to change and have more favorable kinetics than IgG.
- Sjogren's syndrome
- immunoglobulin free light chain
- B cells
- plasma cells
- disease activity
- biologic therapies
- LYMPHOID-TISSUE LYMPHOMA
- B-CELL HYPERACTIVITY
- MONOCLONAL GAMMOPATHY
- RITUXIMAB TREATMENT
- ABATACEPT TREATMENT