Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease

Abstract The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, a...

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Autores principales:	Eduardo Martín-Nares, Vanessa Saavedra-González, Reynerio Fagundo-Sierra, Blanca Estela Santinelli-Núñez, Teresa Romero-Maceda, Karla Calderón-Vasquez, Gabriela Hernandez-Molina
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/4e683c93b3454059b4f0deb632a8721e
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spelling	oai:doaj.org-article:4e683c93b3454059b4f0deb632a8721e2021-12-02T15:23:29ZSerum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease10.1038/s41598-021-81321-52045-2322https://doaj.org/article/4e683c93b3454059b4f0deb632a8721e2021-01-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-81321-5https://doaj.org/toc/2045-2322Abstract The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry. sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) of IgG4-RD patients, respectively. There were no differences in sFLC among IgG4-RD phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher κ sFLC and λ sFLC. The AUC for κ sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for κ sFLC and κ/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels. sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.Eduardo Martín-NaresVanessa Saavedra-GonzálezReynerio Fagundo-SierraBlanca Estela Santinelli-NúñezTeresa Romero-MacedaKarla Calderón-VasquezGabriela Hernandez-MolinaNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Eduardo Martín-Nares Vanessa Saavedra-González Reynerio Fagundo-Sierra Blanca Estela Santinelli-Núñez Teresa Romero-Maceda Karla Calderón-Vasquez Gabriela Hernandez-Molina Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
description	Abstract The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry. sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) of IgG4-RD patients, respectively. There were no differences in sFLC among IgG4-RD phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher κ sFLC and λ sFLC. The AUC for κ sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for κ sFLC and κ/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels. sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.
format	article
author	Eduardo Martín-Nares Vanessa Saavedra-González Reynerio Fagundo-Sierra Blanca Estela Santinelli-Núñez Teresa Romero-Maceda Karla Calderón-Vasquez Gabriela Hernandez-Molina
author_facet	Eduardo Martín-Nares Vanessa Saavedra-González Reynerio Fagundo-Sierra Blanca Estela Santinelli-Núñez Teresa Romero-Maceda Karla Calderón-Vasquez Gabriela Hernandez-Molina
author_sort	Eduardo Martín-Nares
title	Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
title_short	Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
title_full	Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
title_fullStr	Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
title_full_unstemmed	Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease
title_sort	serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with igg4-related disease
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/4e683c93b3454059b4f0deb632a8721e
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Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease

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