Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients
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...
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Wiley
2018-01-01
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| Series: | Journal of Immunology Research |
| Online Access: | http://dx.doi.org/10.1155/2018/9646209 |
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| author | Umberto Basile Mariapaola Marino Cecilia Napodano Krizia Pocino Paolo Emilio Alboini Francesca Gulli Amelia Evoli Carlo Provenzano Emanuela Bartoccioni |
| author_facet | Umberto Basile Mariapaola Marino Cecilia Napodano Krizia Pocino Paolo Emilio Alboini Francesca Gulli Amelia Evoli Carlo Provenzano Emanuela Bartoccioni |
| author_sort | Umberto Basile |
| collection | DOAJ |
| description | 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 κ chains in both AChR- and MuSK-MG patients, while free λ chain levels were increased only in AChR-MG. We also observed a significant reduction of both free κ and λ 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. |
| format | Article |
| id | doaj-art-7800774ef15747ad99d271f430c29ae2 |
| institution | Kabale University |
| issn | 2314-8861 2314-7156 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Immunology Research |
| spelling | doaj-art-7800774ef15747ad99d271f430c29ae22025-02-03T01:06:40ZengWileyJournal of Immunology Research2314-88612314-71562018-01-01201810.1155/2018/96462099646209Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis PatientsUmberto Basile0Mariapaola Marino1Cecilia Napodano2Krizia Pocino3Paolo Emilio Alboini4Francesca Gulli5Amelia Evoli6Carlo Provenzano7Emanuela Bartoccioni8Dipartimento di Medicina di Laboratorio, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyIstituto di Patologia Generale, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyDipartimento di Medicina di Laboratorio, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyDipartimento di Medicina di Laboratorio, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyIstituto di Neurologia, Dipartimento di Geriatria, Neuroscienze e Ortopedia, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyDipartimento di Medicina di Laboratorio, Ospedale Madre Giuseppina Vannini, Rome, ItalyIstituto di Neurologia, Dipartimento di Geriatria, Neuroscienze e Ortopedia, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyIstituto di Patologia Generale, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyDipartimento di Medicina di Laboratorio, Fondazione Policlinico Universitario Agostino Gemelli, Università Cattolica del Sacro Cuore, Rome, ItalyBackground. 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 κ chains in both AChR- and MuSK-MG patients, while free λ chain levels were increased only in AChR-MG. We also observed a significant reduction of both free κ and λ 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.http://dx.doi.org/10.1155/2018/9646209 |
| spellingShingle | Umberto Basile Mariapaola Marino Cecilia Napodano Krizia Pocino Paolo Emilio Alboini Francesca Gulli Amelia Evoli Carlo Provenzano Emanuela Bartoccioni Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients Journal of Immunology Research |
| title | Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients |
| title_full | Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients |
| title_fullStr | Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients |
| title_full_unstemmed | Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients |
| title_short | Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients |
| title_sort | serological immunoglobulin free light chain profile in myasthenia gravis patients |
| url | http://dx.doi.org/10.1155/2018/9646209 |
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