Diagnostic value of IgG4 Indices in IgG4-related hypertrophic pachymeningitis

J Neuroimmunol. 2014 Jan 15;266(1-2):82-6. doi: 10.1016/j.jneuroim.2013.10.008. Epub 2013 Oct 22.


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.

Keywords: Cerebrospinal fluid; Hypertrophic pachymeningitis; IgG4; IgG4-related disease; IgG4-related pachymeningitis; Pachymeningitis.

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Hypertrophy
  • Immunoglobulin G / blood*
  • Immunoglobulin G / cerebrospinal fluid*
  • Male
  • Meningitis / blood
  • Meningitis / cerebrospinal fluid
  • Meningitis / diagnosis*
  • Meningitis / immunology*
  • Middle Aged
  • Retrospective Studies


  • Immunoglobulin G