Michael Levraut, Romain Marignier, Mikael Cohen, Jeanne Benoit, Cassandre Landes-Chateau, Elisabeth Maillart, Marion Cremoni, Barbara Seitz-Polski, Anne-Laurie Pinto, Pauline Dumez, Jérôme Honnorat, Christine Lebrun-Frenay
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This study assessed the κ-FLC and λ-FLC indices in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>This multicenter study compares κ-FLC and λ-FLC indexes among patients with autoimmune GFAP astrocytopathy and sex- and age-matched MS (positive control group) as well as symptomatic controls (headaches and small cerebral vessel disease, as the negative control group). We describe the correlation of both indexes with clinical variables and outcomes in the GFAP astrocytopathy cohort.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>A total of 93 patients were included (31 in each group). The median κ-FLC index was higher in the MS group (65.5 [35.7; 118.3]) compared to the GFAP astrocytopathy group (26.1 [11.4; 78.4], <i>p</i> = 0.062). With a κ-FLC index threshold of 6.1, the proportion of patients with a positive κ-FLC index was similar between the MS (94%) and GFAP-astrocytopathy groups (84%, <i>p</i> = 0.425). The median λ-FLC index was higher in the GFAP astrocytopathy group (45.5 [28.4; 96.9]) than in the MS group (10.6 [2.2; 29.1], <i>p</i> < 0.001).</p>\n \n <p>In the GFAP-astrocytopathy group, both CSF λ-FLC and the λ-FLC index at baseline were correlated with the last follow-up mRS (<i>⍴</i> = 0.46, <i>r</i><sup>2</sup> = 0.088, <i>p</i> = 0.014, and <i>⍴</i> = 0.32, <i>r</i><sup>2</sup> = 0.12, <i>p</i> = 0.101, respectively).</p>\n </section>\n \n <section>\n \n <h3> Conclusion</h3>\n \n <p>The κ-FLC index alone cannot distinguish between autoimmune GFAP astrocytopathy and MS. We indicate a potential diagnostic and prognostic role of the λ-FLC index in GFAP astrocytopathy that needs confirmation in independent cohorts.</p>\n </section>\n </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 9","pages":"1785-1793"},"PeriodicalIF":3.9000,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.70103","citationCount":"0","resultStr":"{\"title\":\"Relevance of Kappa and Lambda Free Light Chains in Autoimmune Astrocytopathy Associated With Anti-GFAP Antibodies\",\"authors\":\"Michael Levraut, Romain Marignier, Mikael Cohen, Jeanne Benoit, Cassandre Landes-Chateau, Elisabeth Maillart, Marion Cremoni, Barbara Seitz-Polski, Anne-Laurie Pinto, Pauline Dumez, Jérôme Honnorat, Christine Lebrun-Frenay\",\"doi\":\"10.1002/acn3.70103\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Introduction</h3>\\n \\n <p>The kappa-free light chain (κ-FLC) index is known to be highly sensitive and specific for diagnosing multiple sclerosis (MS), while little is understood about lambda (λ)-FLC. This study assessed the κ-FLC and λ-FLC indices in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Methods</h3>\\n \\n <p>This multicenter study compares κ-FLC and λ-FLC indexes among patients with autoimmune GFAP astrocytopathy and sex- and age-matched MS (positive control group) as well as symptomatic controls (headaches and small cerebral vessel disease, as the negative control group). We describe the correlation of both indexes with clinical variables and outcomes in the GFAP astrocytopathy cohort.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Results</h3>\\n \\n <p>A total of 93 patients were included (31 in each group). The median κ-FLC index was higher in the MS group (65.5 [35.7; 118.3]) compared to the GFAP astrocytopathy group (26.1 [11.4; 78.4], <i>p</i> = 0.062). With a κ-FLC index threshold of 6.1, the proportion of patients with a positive κ-FLC index was similar between the MS (94%) and GFAP-astrocytopathy groups (84%, <i>p</i> = 0.425). The median λ-FLC index was higher in the GFAP astrocytopathy group (45.5 [28.4; 96.9]) than in the MS group (10.6 [2.2; 29.1], <i>p</i> < 0.001).</p>\\n \\n <p>In the GFAP-astrocytopathy group, both CSF λ-FLC and the λ-FLC index at baseline were correlated with the last follow-up mRS (<i>⍴</i> = 0.46, <i>r</i><sup>2</sup> = 0.088, <i>p</i> = 0.014, and <i>⍴</i> = 0.32, <i>r</i><sup>2</sup> = 0.12, <i>p</i> = 0.101, respectively).</p>\\n </section>\\n \\n <section>\\n \\n <h3> Conclusion</h3>\\n \\n <p>The κ-FLC index alone cannot distinguish between autoimmune GFAP astrocytopathy and MS. 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引用次数: 0
摘要
摘要：kappa-free light chain （κ-FLC）指数对于多发性硬化症（MS）的诊断具有很高的敏感性和特异性，而lambda (λ)-FLC的诊断却知之甚少。本研究评估自身免疫胶质纤维酸性蛋白（GFAP）星形细胞病中κ-FLC和λ-FLC指数的变化。方法：本多中心研究比较自身免疫性GFAP星形细胞病和性别、年龄匹配的MS患者（阳性对照组）和症状对照组（头痛、小脑血管病为阴性对照组）的κ-FLC和λ-FLC指标。我们描述了GFAP星形细胞病变队列中这两个指标与临床变量和结果的相关性。结果：共纳入93例患者，每组31例。MS组中位κ-FLC指数较高(65.5 [35.7；118.3])与GFAP星形细胞病变组相比(26.1 [11.4；[78.4], p = 0.062)。在κ-FLC指数阈值为6.1的情况下，MS组和gmap -星形细胞病变组中κ-FLC指数阳性的患者比例相近（94%），两者差异无统计学意义（84%,p = 0.425）。GFAP星形细胞病变组中位λ-FLC指数较高(45.5 [28.4；96.9])高于MS组(10.6 [2.2；29.1], p 2 = 0.088, p = 0.014,⍴= 0.32,r2 = 0.12, p = 0.101)。结论：单靠κ-FLC指数不能区分自身免疫性GFAP星形细胞病和ms。我们提示，λ-FLC指数在GFAP星形细胞病中具有潜在的诊断和预后作用，这需要在独立的队列中得到证实。
Relevance of Kappa and Lambda Free Light Chains in Autoimmune Astrocytopathy Associated With Anti-GFAP Antibodies
Introduction
The kappa-free light chain (κ-FLC) index is known to be highly sensitive and specific for diagnosing multiple sclerosis (MS), while little is understood about lambda (λ)-FLC. This study assessed the κ-FLC and λ-FLC indices in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.
Methods
This multicenter study compares κ-FLC and λ-FLC indexes among patients with autoimmune GFAP astrocytopathy and sex- and age-matched MS (positive control group) as well as symptomatic controls (headaches and small cerebral vessel disease, as the negative control group). We describe the correlation of both indexes with clinical variables and outcomes in the GFAP astrocytopathy cohort.
Results
A total of 93 patients were included (31 in each group). The median κ-FLC index was higher in the MS group (65.5 [35.7; 118.3]) compared to the GFAP astrocytopathy group (26.1 [11.4; 78.4], p = 0.062). With a κ-FLC index threshold of 6.1, the proportion of patients with a positive κ-FLC index was similar between the MS (94%) and GFAP-astrocytopathy groups (84%, p = 0.425). The median λ-FLC index was higher in the GFAP astrocytopathy group (45.5 [28.4; 96.9]) than in the MS group (10.6 [2.2; 29.1], p < 0.001).
In the GFAP-astrocytopathy group, both CSF λ-FLC and the λ-FLC index at baseline were correlated with the last follow-up mRS (⍴ = 0.46, r2 = 0.088, p = 0.014, and ⍴ = 0.32, r2 = 0.12, p = 0.101, respectively).
Conclusion
The κ-FLC index alone cannot distinguish between autoimmune GFAP astrocytopathy and MS. We indicate a potential diagnostic and prognostic role of the λ-FLC index in GFAP astrocytopathy that needs confirmation in independent cohorts.
期刊介绍：
Annals of Clinical and Translational Neurology is a peer-reviewed journal for rapid dissemination of high-quality research related to all areas of neurology. The journal publishes original research and scholarly reviews focused on the mechanisms and treatments of diseases of the nervous system; high-impact topics in neurologic education; and other topics of interest to the clinical neuroscience community.
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