title: Evidence for peripheral immune activation in amyotrophic lateral sclerosis.
abstract: There is evidence of the activity of immune system in the spinal cords of patients with amyotrophic lateral sclerosis (ALS), however; few studies to date have explored the status of peripheral immune response in ALS patients. Blood samples from 284 ALS patients and 217 aged-match controls were evaluated, and parameters of T cell subset, humoral immunity, and complement system activation were observed. CD4+ T lymphocytes and circulating immune complexes (CICs) were significantly decreased, and component C3 was significantly increased in ALS patients compared with normal controls. Patients with severe or moderate impairment had a higher CD4+ T cell percentage and a lower IgG levels when compared to those with mild impairment. There was an inverse correlation between CD4 T cell percentage and both revised ALS Functional Rating Scale (ALSFRS-R) score and disease duration, but the correlation was positive between IgG level and both ALSFRS-R score and disease duration among ALS patients. These correlations were gender-specific. This investigation demonstrated the existence of peripheral immune abnormalities in ALS patients.
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Xueping
authors.initials: X
authors.lastname: Chen
authors.affiliation: Division of Clinical Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Weihua
authors.initials: W
authors.lastname: Feng
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Huang
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Xiaoyan
authors.initials: X
authors.lastname: Guo
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Yongping
authors.initials: Y
authors.lastname: Chen
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
authors.forename: Zhenzhen
authors.initials: Z
authors.lastname: Zheng
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. Electronic address: hfshang2002@126.com.
authors.forename: Huifang
authors.initials: H
authors.lastname: Shang
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000276:immunology* / Q000503:physiopathology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D016022:Case-Control Studies; D003176:Complement C3 / Q000276:immunology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012720:Severity of Illness Index
chemical_list: D003176:Complement C3; D007074:Immunoglobulin G
keywords: Amyotrophic lateral sclerosis; CD4+ T lymphocytes; Disease duration; IgG; Peripheral immune activation; Revised ALS Functional Rating Scale (ALSFRS-R)