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Cerebrospinal Fluid Analysis in Immunoglobulin G4-related Hypertrophic Pachymeningitis

Published on Nov 1, 2013in The Journal of Rheumatology 3.63
· DOI :10.3899/jrheum.130678
Emanuel Della-Torre13
Estimated H-index: 13
(UniSR: Vita-Salute San Raffaele University),
Gabriella Passerini8
Estimated H-index: 8
(UniSR: Vita-Salute San Raffaele University)
+ 7 AuthorsDiego Franciotta39
Estimated H-index: 39
Cite
Abstract
To the Editor: Immunoglobulin G4-related disease (IgG4-RD) is characterized by fibrous swelling of affected organs, elevations in serum IgG4 concentrations, and responsiveness to glucocorticoid treatment1. Affected tissues display similar histological features: diffuse lymphoplasmacytic infiltration by numerous IgG4-positive plasma cells, occasional eosinophils, storiform fibrosis, and obliterative phlebitis2. IgG4-related hypertrophic pachymeningitis (IgG4-HP) has been identified as a characteristic central nervous system (CNS) manifestation of IgG4-RD, but comprehensive cerebrospinal fluid (CSF) analyses are substantially lacking3,4. Recently, we demonstrated an intrathecal IgG and IgG4 synthesis in the CSF of a patient with IgG4-HP and suggested IgG4 Indices as safe potential diagnostic tools for IgG4-HP5. Here, we describe 2 new cases of IgG4-HP with CSF evaluation at diagnosis and in response to treatment. In case 1, March 2007, a 56-year-old man was admitted with a 1-year history of right frontal headache. Magnetic resonance imaging (MRI) showed right frontal pachymeningitis. Granulomatous meningeal inflammation was suspected and oral prednisone (1 mg/kg body weight/day) was started with clinical and radiological improvement. Prednisone was discontinued after 8 months, but in July 2008 the headache recurred. Blood and CSF analyses are shown in Table 1. MRI evidenced a right frontotemporal progression of the pachymeningitis (Figure 1A). A meningeal biopsy … Address correspondence to Dr. E. Della-Torre, Department of Medicine and Clinical Immunology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. E-mail: dellatorre.emanuel{at}hsr.it
  • References (11)
  • Citations (24)
Cite
References11
Newest
Published on Dec 1, 2012in Journal of Immunology 4.72
Oliver Winter7
Estimated H-index: 7
(Charité),
Christof Dame23
Estimated H-index: 23
(Charité)
+ 1 AuthorsFalk Hiepe52
Estimated H-index: 52
(Charité)
Long-lived plasma cells survive in a protected microenvironment for years or even a lifetime and provide humoral memory by establishing persistent Ab titers. Long-lived autoreactive, malignant, and allergen-specific plasma cells are likewise protected in their survival niche and are refractory to immunosuppression, B cell depletion, and irradiation. Their elimination remains an essential therapeutic challenge. Recent data indicate that long-lived plasma cells reside in a multicomponent plasma ce...
Published on Sep 1, 2012in Modern Pathology 6.37
Vikram V. Deshpande85
Estimated H-index: 85
(Harvard University),
Yoh Zen62
Estimated H-index: 62
(University of Cambridge)
+ 38 AuthorsJudith A. Ferry57
Estimated H-index: 57
(Harvard University)
IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; and-often but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Ital...
Published on Mar 6, 2012in Annals of Internal Medicine 19.32
Thomas S. Huddle10
Estimated H-index: 10
,
Robert M. Centor34
Estimated H-index: 34
Published on Mar 6, 2012in Annals of Internal Medicine 19.32
Emanuel Della Torre3
Estimated H-index: 3
(UniSR: Vita-Salute San Raffaele University),
E. Bozzolo17
Estimated H-index: 17
(UniSR: Vita-Salute San Raffaele University)
+ 2 AuthorsMaria Grazia Sabbadini35
Estimated H-index: 35
(UniSR: Vita-Salute San Raffaele University)
Published on Feb 9, 2012in The New England Journal of Medicine 70.67
John H. Stone67
Estimated H-index: 67
,
Yoh Zen62
Estimated H-index: 62
,
Deshpande5
Estimated H-index: 5
Published on Jan 1, 2012in International Journal of Rheumatology
Mollie N. Carruthers15
Estimated H-index: 15
(Partners HealthCare),
John H. Stone67
Estimated H-index: 67
(Partners HealthCare)
+ 1 AuthorsArezou Khosroshahi25
Estimated H-index: 25
IgG4-related disease (IgG4-RD) is a multiorgan inflammatory disease in which diverse organ manifestations are linked by common histopathological and immunohistochemical features. Prospective studies of IgG4-RD patients are required to clarify the natural history, long-term prognosis, and treatment approaches in this recently recognized condition. Patients with IgG4-RD have different organ manifestations and are followed by multiple specialties. Divergent approaches to the assessment of patients ...
Published on Sep 1, 2010in Advances in Anatomic Pathology 3.53
Wah Cheuk30
Estimated H-index: 30
,
John K. C. Chan79
Estimated H-index: 79
An elevated serum titer of immunoglobulin G4 (IgG4), the least common (3% to 6%) of the 4 subclasses of IgG, is a surrogate marker for the recently characterized IgG4-related sclerosing disease. The syndrome affects predominantly middle-aged and elderly patients, with male predominance. The patients
Published on Aug 1, 2009in The American Journal of Surgical Pathology 6.16
Siu-Ki Chan1
Estimated H-index: 1
,
Wah Cheuk30
Estimated H-index: 30
+ 1 AuthorsJohn K. C. Chan79
Estimated H-index: 79
Abstract IgG4-related sclerosing disease is a distinctive mass-forming lesion with frequent systemic involvement, most frequently the pancreas, salivary glands, and lacrimal glands. This report describes a case manifesting with a previously unrecognized form of central nervous system involvement. The 37-year-old man presented with signs and symptoms of spinal cord compression at the thoracic level 9. Magnetic resonance imaging revealed an elongated dural mass extending from the fifth to tenth th...
Published on Sep 1, 2008in Lancet Neurology 28.75
Diego Franciotta39
Estimated H-index: 39
,
Marco Salvetti51
Estimated H-index: 51
(Sapienza University of Rome)
+ 2 AuthorsFrancesca Aloisi53
Estimated H-index: 53
(ISS: Istituto Superiore di Sanità)
Summary Clonal expansion of B cells and the production of oligoclonal IgG in the brain and cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS) have long been interpreted as circumstantial evidence of the immune-mediated pathogenesis of the disease and suggest a possible infectious cause. Extensive work on intrathecally produced antibodies has not yet clarified whether they are pathogenetically relevant. Irrespective of antibody specificity, however, the processes of antibody synth...
Published on Mar 1, 2001in Journal of the Neurological Sciences 2.65
Hansotto Reiber27
Estimated H-index: 27
,
James B. Peter1
Estimated H-index: 1
Abstract Cerebrospinal fluid (CSF) analysis is a basic tool for diagnosis of neurological diseases. Knowledge regarding blood–CSF barrier function (molecular flux/CSF flow theory) and neuroimmunology is reviewed to aid understanding and evaluation of CSF data. Disease-related immunoglobulin patterns (IgG, IgA, IgM with reference to albumin) are described in CSF/serum quotient diagrams with the hyperbolic reference range for blood-derived protein fractions in CSF. Clinical relevance of complement...
Cited By24
Newest
Published on Jun 1, 2019in World Neurosurgery 1.72
Stephen J. Slade (Virginia Mason Medical Center), Erin M. Bauer (Virginia Mason Medical Center)+ 1 AuthorsAmish J. Dave (Virginia Mason Medical Center)
Abstract Background Back pain is a leading reason patients seek medical attention. While musculoskeletal causes are common, rarer etiologies can present. Case Description A 50-year-old male presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. Over the next 3 months, his pain worsened, and he developed lower extremity dysesthesias and subjective weakness despite a normal neurological exam. Non-revealing laboratory workup included norm...
Published on Mar 4, 2019in Expert Review of Clinical Immunology 3.91
Kazunori Yamada15
Estimated H-index: 15
(Kanazawa University),
Ichiro Mizushima11
Estimated H-index: 11
(Kanazawa University),
Mitsuhiro Kawano29
Estimated H-index: 29
(Kanazawa University)
ABSTRACTIntroduction: Recently, IgG4-related disease (IgG4-RD) has become a well-recognized clinical entity, although its causes are still not well understood. The pathophysiology of IgG4-RD has been reported from a variety of aspects.Areas covered: In this review, we outline a number of recent advances in our understanding of the pathogenesis of IgG4-RD, divided according to acquired immunology and innate immunology and other topics. Furthermore, we also focus on some proposed markers of diseas...
Published on Jan 1, 2019
Kristie M. Smith (BIDMC: Beth Israel Deaconess Medical Center), Robert H. Shmerling23
Estimated H-index: 23
(BIDMC: Beth Israel Deaconess Medical Center)
In this chapter, laboratory tests relevant to major disorders in rheumatology that can affect the nervous system are discussed. While laboratory tests can be helpful in evaluating patients with suspected or established rheumatic disease, they are often not diagnostic due to imperfect sensitivity and specificity. Therefore, it is particularly important to recognize that the pretest probability of disease is the driving factor in determining the utility of any laboratory value. Laboratory testing ...
Published on Jul 1, 2018in Neuroradiology 2.50
Jasmina Boban2
Estimated H-index: 2
(University of Novi Sad),
Selin Ardalı2
Estimated H-index: 2
(Hacettepe University),
Majda M. Thurnher27
Estimated H-index: 27
(Medical University of Vienna)
Purpose Immunoglobulin G4 (IgG4)-related disease represents a spectrum of fibro-inflammatory disorders that affects various organ systems, including the central nervous system.
Published on Nov 1, 2017in International Journal of Rheumatic Diseases 1.94
Andreu Fernández Codina7
Estimated H-index: 7
(Autonomous University of Barcelona),
Manuel Hernández-González3
Estimated H-index: 3
(Autonomous University of Barcelona)
+ 3 AuthorsFernando Martínez-Valle7
Estimated H-index: 7
(Autonomous University of Barcelona)
Published on Sep 28, 2017in British Journal of Neurosurgery 1.48
Kelly J. Bridges2
Estimated H-index: 2
(OHSU: Oregon Health & Science University),
Caitlin H. DeDeaux (OHSU: Oregon Health & Science University), Khoi D. Than5
Estimated H-index: 5
(OHSU: Oregon Health & Science University)
AbstractObject: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder affecting various anatomical sites, and only recently was identified to affect the dura of the spine. The authors present the second reported case of an intradural extramedullary lesion consistent with IgG4-related spinal disease.Methods: A literature review was performed that identified 15 other cases of spinal disease, and common features of all known reported spinal IgG4-RD are discussed.Results: Spinal IgG4-RD ty...
Ke Zheng1
Estimated H-index: 1
(Peking Union Medical College Hospital),
Fei Teng1
Estimated H-index: 1
(Peking Union Medical College Hospital),
Xue-Mei Li1
Estimated H-index: 1
(Peking Union Medical College Hospital)
Abstract Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (...
Published on May 1, 2017in European Spine Journal 2.51
Ruofu Tang1
Estimated H-index: 1
(ZJU: Zhejiang University),
Fangcai Li12
Estimated H-index: 12
(ZJU: Zhejiang University),
Qixin Chen12
Estimated H-index: 12
(ZJU: Zhejiang University)
Background Thoracic spinal stenosis caused by ossification of ligamentum flavum (OLF) is frequently seen, but long segmental thoracic spinal cord compressed by consequent thickened ligament posteriorly was rarely reported.
Published on Apr 1, 2017in Current Treatment Options in Neurology 3.57
Bernardo Baptista1
Estimated H-index: 1
,
Alina Casian6
Estimated H-index: 6
(Guy's and St Thomas' NHS Foundation Trust)
+ 2 AuthorsClaire M Rice2
Estimated H-index: 2
(Southmead Hospital)
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG...