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.

Basile, U., Marino, M., Napodano, C., Pocino, K., Alboini, P. E., Gulli, F., Evoli Stampanoni-B, A., Provenzano, C., Bartoccioni, E., Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients, <<JOURNAL OF IMMUNOLOGY RESEARCH>>, 2018; 2018 (Mar 25): N/A-N/A. [doi:10.1155/2018/9646209] [http://hdl.handle.net/10807/119741]

Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients

Basile, Umberto
Co-primo
;
Marino, Mariapaola
Co-primo
;
Napodano, Cecilia;Pocino, Krizia;Alboini, Paolo Emilio;Gulli, Francesca;Evoli Stampanoni-B, Amelia;Provenzano, Carlo
Penultimo
;
Bartoccioni, Emanuela
Ultimo
2018

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.
2018
Inglese
Basile, U., Marino, M., Napodano, C., Pocino, K., Alboini, P. E., Gulli, F., Evoli Stampanoni-B, A., Provenzano, C., Bartoccioni, E., Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients, <<JOURNAL OF IMMUNOLOGY RESEARCH>>, 2018; 2018 (Mar 25): N/A-N/A. [doi:10.1155/2018/9646209] [http://hdl.handle.net/10807/119741]
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/119741
Citazioni
  • ???jsp.display-item.citation.pmc??? 7
  • Scopus 21
  • ???jsp.display-item.citation.isi??? 20
social impact