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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)
Sources
Abstract
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.
  • References (15)
  • Citations (29)
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References15
Newest
Nov 1, 2013·The Journal of Rheumatology3.63
#1Emanuel Della-Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 17
#2Gabriella Passerini (UniSR: Vita-Salute San Raffaele University)H-Index: 11
Last. Diego FranciottaH-Index: 42
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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 character...
26 CitationsSource
Jul 1, 2013·Medicine1.87
#1Zachary S. WallaceH-Index: 21
#2Mollie N. CarruthersH-Index: 15
Last. John H. StoneH-Index: 72
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91 CitationsSource
Jun 1, 2013·Hepatology14.97
#1Lucas Maillette de Buy WennigerH-Index: 16
#2Marieke E. Doorenspleet (UvA: University of Amsterdam)H-Index: 15
Last. Ulrich BeuersH-Index: 60
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Immunoglobulin G4 (IgG4)-associated cholangitis (IAC) is a manifestation of the recently discovered idiopathic IgG4-related disease. The majority of patients have elevated serum IgG4 levels and/or IgG4-positive B-cell and plasma cell infiltrates in the affected tissue. We hypothesized that clonally expanded, class-switched IgG4-positive B cells and plasma cells could be causal to these poorly understood phenomena. In a prospective cohort of six consecutive IAC patients, six healthy controls, and...
90 CitationsSource
Sep 27, 2012·Pathology Research International
#1Fereydoun Davatchi (Tehran University of Medical Sciences)H-Index: 32
Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For IC...
61 CitationsSource
Sep 1, 2012·Modern Pathology6.37
#1Vikram Deshpande (Harvard University)H-Index: 93
#2Yoh Zen (University of Cambridge)H-Index: 65
Last. John H. Stone (Harvard University)H-Index: 72
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Aug 1, 2012·American Journal of Hematology6.14
#1Angela Dispenzieri (UR: University of Rochester)H-Index: 101
Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the sy...
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Mar 6, 2012·Annals of Internal Medicine19.32
#1Emanuel Della Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 3
#2Enrica Bozzolo (UniSR: Vita-Salute San Raffaele University)H-Index: 18
Last. Maria Grazia Sabbadini (UniSR: Vita-Salute San Raffaele University)H-Index: 36
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32 CitationsSource
Purpose of review To review studies that have examined underlying genetic and immunological aspects of IgG4-related disease. Recent findings Genetic studies have suggested that several human leukocyte antigen (HLA) and non-HLA haplotypes/genotypes are associated with susceptibility to IgG4-related disease or to disease relapse after steroid therapy. Among several autoantibodies identified so far, autoantibodies against lactoferrin and carbonic anhydrase II are most frequently detected in serum o...
104 CitationsSource
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
296 CitationsSource
Aug 11, 2006·Annals of the Rheumatic Diseases14.30
#1Richard A. Watts (UEA: University of East Anglia)H-Index: 50
#2SE LaneH-Index: 15
Last. David G. I. Scott (Norwich University)H-Index: 34
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Background: The classification of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN) for epidemiology studies is confusing. The existing schemes such as American College of Rheumatology (ACR) criteria, Chapel Hill Consensus Conference (CHCC) definitions and Lanham criteria produce overlapping and conflicting classifications, making it difficult to compare incidence figures. Aim: To develop a consensus method of using these criteria and definitions for ...
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#1J-Matthias Löhr (Karolinska University Hospital)H-Index: 9
#2Ulrich Beuers (UvA: University of Amsterdam)H-Index: 60
Last. Emanuel Della-Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 17
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The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, ...
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Jun 16, 2020·BMJ27.60
#1Marco Lanzillotta (UniSR: Vita-Salute San Raffaele University)H-Index: 10
#2Gaia Mancuso (UniSR: Vita-Salute San Raffaele University)H-Index: 1
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ABSTRACT IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multif...
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Background Back pain is a leading reason for patients to seek medical attention. Although musculoskeletal causes are common, patients can also present with rarer etiologies. Case Description A 50-year-old man presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. During the next 3 months, his pain worsened, and he developed lower extremity dysesthesia and subjective weakness, despite normal neurological examination findings. Nonreveali...
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Objective Meningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP). Methods Two IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and cas...
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#2M. Chand Sharma (AIIMS: All India Institute of Medical Sciences)
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Background: IgG4-related disease is an autoimmune process that presents with tumefactive lesions characterized by storiform fibrosis, a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, obliterative phlebitis, and often elevated serum IgG4 levels. Central nervous system IgG4-related disease is very rare and usually occurs in the form of hypertrophic pachymeningitis or hypophysitis. Presentation as a large solitary meningioma-like mass with overlying hyperostosis in a young adult has...
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: Immunoglobulin G4-related disease (IgG4-RD) is an uncommon disorder that demonstrates characteristic clinicopathologic features including sclerosing lesions with storiform fibrosis, increased IgG4+ plasma cells with an increased IgG4+/IgG+ plasma cell ratio, obliterative phlebitis, and often an increased serum IgG4 level. This review summarizes the characteristic histopathologic and clinical features of IgG4-RD with detailed discussion of the histopathologic characteristics of the most commonl...
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A 65-year-old man working as a car dealer was referred to our neurology outpatient clinic because of a pituitary mass. Two years before referral, the patient had developed symptoms of polyuria and polydipsia, decreased endurance while working out, fatigue, loss of body hair, and erectile dysfunction. The endocrinologist found hypogonadotropic hypogonadism, hypocortisolism, and central diabetes insipidus, which was treated with testosterone, hydrocortisone, and desmopressin, resulting in adequate...
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BackgroundHemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms...
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