Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients

Mariapaola Marino, Amelia Evoli, Carlo Provenzano, Umberto Basile, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Emanuela Bartoccioni

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Background. Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability. As MG course may be remarkably variable, we evaluated the possible use of FLCs as biomarkers of disease activity. Subjects and Methods. We assessed FLC levels in 34 sera from 17 AChR-MG and from 13 MuSK-MG patients, in comparison with 20 sera from patients with systemic autoimmune rheumatic diseases and 18 from healthy blood donors, along with titers of specific auto-abs and IgG subclass distribution. Results. We found a statistically significant increase in free. chains in both AChR-and MuSK-MG patients, while free lambda chain levels were increased only in AChR-MG. We also observed a significant reduction of both free kappa and lambda chains in 1/4 MuSK-MG patients along with specific abs titer, two months after RTX treatment. Conclusions. From our data, FLCs appear to be a sensitive marker of B cell activation in MG. Further investigations are necessary to exploit their potential as reliable biomarkers of disease activity.
Original languageEnglish
Pages (from-to)N/A-N/A
JournalJournal of Immunology Research
Volume2018
DOIs
Publication statusPublished - 2018

Keywords

  • Free Light Chain, autoimmune diseases, Myasthenia gravis, IgG subclasses, autoantibodies, rituximab

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