Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis

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• 作者：Emanuel Della-Torre ; Laura Galli ; Diego Franciotta ; Enrica Paola Bozzolo ; Chiara Briani ; Roberto Furlan ; Luisa Roveri ; Maria Sessa ; Gabriella Passerini ; Maria Grazia Sabbadini
• 关键词：IgG4 ; IgG4-related disease ; IgG4-related pachymeningitis ; Hypertrophic pachymeningitis ; Pachymeningitis ; Cerebrospinal fluid
• 刊名：Journal of Neuroimmunology
• 出版年：15 January, 2014
• 年：2014
• 卷：266
• 期：1-2
• 页码：82-86
• 全文大小：343 K

文摘

Diagnosis of IgG4-Related Hypertrophic Pachymeningitis (IgG4-HP) relies on meningeal biopsies, because cerebrospinal fluid (CSF) diagnostic biomarkers are lacking. Here, we determined whether IgG4 intrathecal production could distinguish IgG4-HP from other disorders presenting with HP (OHP). In patients with IgG4-HP, the median CSF IgG4 concentration, IgG4 Index and IgG4_Loc were significantly higher than in both controls and OHP. CSF IgG4 levels higher than 2.27 mg/dL identified 100% of IgG4-HP and 5% of OHP. An IgG4_Loc cut-off of 0.47 identified 100% of IgG4-HP and no cases of OHP. Our results support CSF IgG4 quantification and IgG4 Indices as alternatives to meningeal biopsy for the diagnosis of IgG4-HP when this procedure is contraindicated or uninformative.