Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis ☆ ☆☆ ★

Published on Jan 1, 2014in Journal of Neuroimmunology2.832
· DOI :10.1016/j.jneuroim.2013.10.008
Emanuel Della-Torre17
Estimated H-index: 17
(UniSR: Vita-Salute San Raffaele University),
Laura Galli1
Estimated H-index: 1
+ 7 AuthorsMaria Grazia Sabbadini36
Estimated H-index: 36
(UniSR: Vita-Salute San Raffaele University)
Abstract 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 2.27 mg/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.
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