Serum immunoglobulin free light chains are sensitive biomarkers for monitoring disease activity and treatment response in primary Sjogren's syndrome

Gwenny M. Verstappen*, Rada Moerman, Jolien F. van Nimwegen, Martha S. van Ginkel, Johan Bijzet, Esther Mossel, Arjan Vissink, Bouke P. C. Hazenberg, Suzanne Arends, Frans G. M. Kroese, Hendrika Bootsma

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

11 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1812-1821
Number of pages10
JournalRheumatology
Volume57
Issue number10
Early online date2-Jul-2018
DOIs
Publication statusPublished - 1-Oct-2018

Keywords

  • Sjogren's syndrome
  • immunoglobulin free light chain
  • lymphoma
  • B cells
  • plasma cells
  • disease activity
  • biomarker
  • biologic therapies
  • rituximab
  • abatacept
  • SYSTEMIC-LUPUS-ERYTHEMATOSUS
  • LYMPHOID-TISSUE LYMPHOMA
  • B-CELL HYPERACTIVITY
  • MONOCLONAL GAMMOPATHY
  • RITUXIMAB TREATMENT
  • ABATACEPT TREATMENT
  • RHEUMATOID-FACTOR
  • CONTROLLED-TRIAL
  • PATHOGENESIS
  • MARKER

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