IgG4-Related Pachymeningitis: Evidence of Intrathecal IgG4 on Cerebrospinal Fluid Analysis
Authors: Emanuel Della Torre, Enrica Paola Bozzolo, Gabriella Passerini, Claudio Doglioni, Maria Grazia Sabbadini
Journal: Annals of Internal Medicine (2012)

Letters6 March 2012IgG4-Related Pachymeningitis: Evidence of Intrathecal IgG4 on Cerebrospinal Fluid AnalysisEmanuel Della Torre, MD, Enrica Paola Bozzolo, MD, Gabriella Passerini, MD, Claudio Doglioni, MD, and Maria Grazia Sabbadini, MDEmanuel Della Torre, MDFrom San Raffaele Scientific Institute, 20132 Milan, Italy.Search for more papers by this author, Enrica Paola Bozzolo, MDFrom San Raffaele Scientific Institute, 20132 Milan, Italy.Search for more papers by this author, Gabriella Passerini, MDFrom San Raffaele Scientific Institute, 20132 Milan, Italy.Search for more papers by this author, Claudio Doglioni, MDFrom San Raffaele Scientific Institute, 20132 Milan, Italy.Search for more papers by this author, and Maria Grazia Sabbadini, MDFrom San Raffaele Scientific Institute, 20132 Milan, Italy.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-156-5-201203060-00025 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Hypertrophic pachymeningitis is a clinical disorder caused by thickening of dura mater from chronic inflammation that is labeled idiopathic in the absence of an identifiable cause. Other investigators recently reported a prominent lymphoplasmacytic infiltration of immunoglobulin G4+ (IgG4) cells in 3 patients with hypertrophic pachymeningitis, suggesting that some idiopathic cases may be part of the IgG4-related systemic disease spectrum (1–4).Objective: To look for IgG4 in cerebrospinal fluid (CSF) from a patient with IgG4-related pachymeningitis.Case Report: In July 2009, a 65-year-old Italian man with autoimmune hypothyroidism reported fever associated with evanescent nodular skin lesions on his arms. Laboratory ...References1. Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009;33:1249-52. [PMID: 19561447] CrossrefMedlineGoogle Scholar2. Kosakai A, Ito D, Yamada S, Ideta S, Ota Y, Suzuki N. A case of definite IgG4-related pachymeningitis. Neurology. 2010;75:1390-2. [PMID: 20938033] CrossrefMedlineGoogle Scholar3. Kato K, Tamano Y, Namioka A, Nakagawa M, Kadoyama S, Ujiie H, et al. [Hypertrophic pachymeningitis associated with autoimmune pancreatitis examined for IgG4 related disease: a case report]. No Shinkei Geka. 2011;39:763-8. [PMID: 21799226] MedlineGoogle Scholar4. Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010;120:765-76. [PMID: 20844883] CrossrefMedlineGoogle Scholar5. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303-32. [PMID: 20733352] CrossrefMedlineGoogle Scholar6. Di Pauli F, Gredler V, Kuenz B, Lutterotti A, Ehling R, Gneiss C, et al. Features of intrathecal immunoglobulins in patients with multiple sclerosis. J Neurol Sci. 2010;288:147-50. [PMID: 19828154] CrossrefMedlineGoogle Scholar7. Greve B, Magnusson CG, Melms A, Weissert R. Immunoglobulin isotypes reveal a predominant role of type 1 immunity in multiple sclerosis. J Neuroimmunol. 2001;121:120-5. [PMID: 11730948] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From San Raffaele Scientific Institute, 20132 Milan, Italy.Acknowledgment: The authors thank Dr. Nicoletta Anzalone (Unit of Neuroradiology, San Raffaele Scientific Institute) and Dr. Paola Mapelli (Department of Nuclear Medicine, San Raffaele Scientific Institute) for radiologic studies and Dr. Roberto Furlan (Neuroimmunology Unit–DIBIT and Department of Neuroscience, San Raffaele Scientific Institute) for helpful input and review of the manuscript.Disclosures: None disclosed. 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