Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis

Emanuel Della-Torre, Laura Galli, Diego Franciotta, Enrica Paola Bozzolo, Chiara Briani, Roberto Furlan, Luisa Roveri, Maria Sessa, Gabriella Passerini, Maria Grazia Sabbadini

Research output: Contribution to journalArticlepeer-review


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 IgG4Loc were significantly higher than in both controls and OHP. CSF IgG4 levels higher than 2.27mg/dL identified 100% of IgG4-HP and 5% of OHP. An IgG4Loc 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.

Original languageEnglish
Pages (from-to)82-86
Number of pages5
JournalJournal of Neuroimmunology
Issue number1-2
Publication statusPublished - Jan 15 2014


  • Cerebrospinal fluid
  • Hypertrophic pachymeningitis
  • IgG4
  • IgG4-related disease
  • IgG4-related pachymeningitis
  • Pachymeningitis

ASJC Scopus subject areas

  • Immunology
  • Clinical Neurology
  • Immunology and Allergy
  • Neurology


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