IgG4-Related Pachymeningitis: Evidence of Intrathecal IgG4 on Cerebrospinal Fluid Analysis

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. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByNodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and PrednisoneIntrathecal activation of CD8 + memory T cells in IgG4‐related disease of the brain parenchymaUtility of the “2019 ACR/EULAR classification criteria” for the management of patients with IgG4-related diseaseIgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two CasesImmunoglobulin G4–Related Hypertrophic Pachymeningitis of the Spine: A Case Report and Systematic Review of the LiteratureIgG4-Related Disease of the Oral Cavity. Case Series from a Large Single-Center Cohort of Italian PatientsAdvances in the diagnosis and management of IgG4 related diseaseClinical presentation, treatment and outcome of IgG4-related pachymeningitis: From a national case registry and literature reviewSpinal IgG4-Related Hypertrophic Pachymeningitis with Spinal Cord Compression: Case Report and Literature ReviewImmunoglobulin G4-related sclerosing cholangitisA New Classification System for IgG4 AutoantibodiesIgG4-related hypertrophic pachymeningitis of the spine with MPO-ANCA seropositivityAlteration of IgG4 levels in cerebrospinal fluid in IgG4-related diseaseImmunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatmentUne pachyméningite révélatrice d’une maladie associée aux IgG4IgG4-related diseaseNeurological Manifestations of IgG4-Related DiseaseIdiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related diseasePathophysiology of IgG4-Related DiseaseIgG4-related disease in Italy: clinical features and outcomes of a large cohort of patientsB-cell depletion attenuates serological biomarkers of fibrosis and myofibroblast activation in IgG4-related diseaseImmunology of IgG4-related diseaseCase of Takayasu arteritis accompanying hypertrophic pachymeningitisL’utilité du FDG-TEP/scan et des autres techniques d’imagerie dans l’évaluation des maladies à IgG4The utility of FDG-PET/CT and other imaging techniques in the evaluation of IgG4-related diseaseIgG4-related midline destructive lesionA nationwide survey of hypertrophic pachymeningitis in JapanAdvantage of 11C-methionine positron emission tomography for assessing IgG4-related central nervous system lesionsA 54 Year-Old Man with Progressive Vision and Hearing LossDiagnostic value of IgG4 Indices in IgG4-Related Hypertrophic PachymeningitisIgG4-Related Intracranial Hypertrophic Pachymeningitis : A Case Report and Review of the LiteratureDiffuse large B-cell lymphoma/follicular lymphoma arising in a background of IgG4-related pachymeningitisCerebrospinal Fluid Analysis in Immunoglobulin G4-related Hypertrophic PachymeningitisA case of multi-system IGG4-related diseaseIgG4-Related Disease and Hypertrophic PachymeningitisIgG4-related Disease Manifesting as Pachymeningitis and Aortitis 6 March 2012Volume 156, Issue 5Page: 401-403KeywordsAntibodiesBiopsyCellsCerebrospinal fluidFibrosisLesionsMeningitisProteinsSystemic diseases ePublished: 6 March 2012 Issue Published: 6 March 2012 CopyrightCopyright © 2012 by American College of Physicians. All Rights Reserved.PDF DownloadLoading ...