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Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis ☆ ☆☆ ★
Published on Jan 1, 2014in Journal of Neuroimmunology 2.65
· DOI :10.1016/j.jneuroim.2013.10.008
Emanuel Della-Torre13
Estimated H-index: 13
 (Vita-Salute San Raffaele University), Laura Galli1
Estimated H-index: 1
 + 7 AuthorsMaria Grazia Sabbadini31
Estimated H-index: 31
 (Vita-Salute San Raffaele University)
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 IgG4 Loc 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 IgG4 Loc 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)
  • Cited By (26)
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References15
Published on Mar 6, 2012in Annals of Internal Medicine 19.38
Emanuel Della Torre3
Estimated H-index: 3
 (Vita-Salute San Raffaele University), Enrica Bozzolo16
Estimated H-index: 16
 (Vita-Salute San Raffaele University)+ 2 AuthorsMaria Grazia Sabbadini31
Estimated H-index: 31
 (Vita-Salute San Raffaele University)
29 Citations Source Cite
Published on Mar 1, 2001in Journal of the Neurological Sciences 2.45
Hansotto Reiber23
Estimated H-index: 23
 , 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...
421 Citations Source Cite
Published on Mar 8, 2001in The New England Journal of Medicine 79.26
Hideaki Hamano36
Estimated H-index: 36
 , Shigeyuki Kawa50
Estimated H-index: 50
 + 8 AuthorsNobuteru Usuda4
Estimated H-index: 4
Background Sclerosing pancreatitis is a unique form of pancreatitis that is characterized by irregular narrowing of the main pancreatic duct, lymphoplasmacytic inflammation of the pancreas, and hypergammaglobulinemia and that responds to glucocorticoid treatment. Preliminary studies suggested that serum IgG4 concentrations are elevated in this disease but not in other diseases of the pancreas or biliary tract. Methods We measured serum IgG4 concentrations using single radial immunodiffusion and ...
1,739 Citations Source Cite
Published on Aug 1, 2012in American Journal of Hematology 5.30
A Dispenzieri27
Estimated H-index: 27
 (University of Rochester)
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...
79 Citations Source Cite
Published on Sep 1, 2010in Advances in Anatomic Pathology 2.36
Wah Cheuk30
Estimated H-index: 30
 , John K. C. Chan75
Estimated H-index: 75
283 Citations Source Cite
Published on Jul 1, 2013in Medicine 2.03
Zachary S. Wallace17
Estimated H-index: 17
 , Mollie N. Carruthers15
Estimated H-index: 15
 + 5 AuthorsJohn H. Stone65
Estimated H-index: 65
Hypertrophic pachymeningitis (HP) is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from mass effect, nerve compression, or vascular compromise. The differential diagnosis of HP includes immune-mediated conditions such as rheumatoid arthritis and vasculitis, malignancies, and infections. Many times, no diagnosis is reached; in such cases, the disease has been described as idiopathic HP. IgG4-related disease (IgG4-RD) i...
75 Citations Source Cite
Published on Nov 1, 2013in The Journal of Rheumatology 3.47
Emanuel Della-Torre13
Estimated H-index: 13
 (Vita-Salute San Raffaele University), Gabriella Passerini8
Estimated H-index: 8
 (Vita-Salute San Raffaele University)+ 7 AuthorsDiego Franciotta37
Estimated H-index: 37
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...
25 Citations Source Cite
Published on Jun 1, 2013in Hepatology 14.08
Lucas Maillette de Buy Wenniger14
Estimated H-index: 14
 , Marieke E. Doorenspleet12
Estimated H-index: 12
 (University of Amsterdam)+ 6 AuthorsUlrich Beuers53
Estimated H-index: 53
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...
81 Citations Source Cite
Published on Jan 1, 2011in Current Opinion in Rheumatology 4.28
Yoh Zen60
Estimated H-index: 60
 , Yasuni Nakanuma76
Estimated H-index: 76
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...
98 Citations Source Cite
Published on Sep 27, 2012in Pathology Research International
Fereydoun Davatchi27
Estimated H-index: 27
 (Tehran University of Medical Sciences)
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...
51 Citations Source Cite
Cited By26
Published on Nov 1, 2017in International Journal of Rheumatic Diseases 2.42
Andreu Fernández-Codina6
Estimated H-index: 6
 (Autonomous University of Barcelona), Manuel Hernández-González3
Estimated H-index: 3
 (Autonomous University of Barcelona)+ 3 AuthorsFernando Martínez-Valle5
Estimated H-index: 5
 (Autonomous University of Barcelona)
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Published on Dec 1, 2014in Journal of the Neurological Sciences 2.45
Mohamad Ezzeldin1
Estimated H-index: 1
 (University of Texas Medical Branch), Ahmad Shawagfeh1
Estimated H-index: 1
 (University of Texas Medical Branch)+ 2 AuthorsXiang Fang25
Estimated H-index: 25
 (University of Texas Medical Branch)
7 Citations Source Cite
Published on Aug 1, 2015in Clinical and Experimental Immunology 3.54
Emanuel Della-Torre13
Estimated H-index: 13
 (Vita-Salute San Raffaele University), M. Lanzillotta4
Estimated H-index: 4
 (Vita-Salute San Raffaele University), Claudio Doglioni73
Estimated H-index: 73
 (Vita-Salute San Raffaele University)
Summary Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4+ plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has remained largely intractable to treatment, many IgG4-RD patients appear to have a condition in which...
68 Citations Source Cite
Published on Jan 1, 2014in Brain Tumor Research and Treatment
Young-Sub Lee1
Estimated H-index: 1
 (Kyungpook National University), Hye Won Lee2
Estimated H-index: 2
 (Kyungpook National University)+ 2 AuthorsJeong-Hyun Hwang7
Estimated H-index: 7
 (Kyungpook National University)
Immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis, defined as focally or diffusely thickened dura mater and lymphoplasmacytic infiltration with increased IgG4 bearing plasma cells, is a rare disease. Moreover, cases involving bone are even rarer. In this report, the authors describe a case of IgG4-related hypertrophic pachymeningitis involving the skull in a 65-year-old man presenting with generalized tonic seizures. There is a 2.4 cm diameter extra-axial mass at the vertex of the le...
7 Citations Source Cite
Published on Mar 1, 2015in Journal of the Neurological Sciences 2.45
Gopal Krishna Dash3
Estimated H-index: 3
 , Bejoy Thomas21
Estimated H-index: 21
 + 1 AuthorsAshalatha Radhakrishnan12
Estimated H-index: 12
Abstract Objective To elucidate the clinico-radiological features, treatment response and outcome of a large cohort of patients (n = 20) with idiopathic hypertrophic pachymeningitis (IHP) and to examine if any of these features could differentiate between IHP and secondary causes of hypertrophic pachymeningitis (SHP). Methods 20 patients with IHP diagnosed between 1998 and 2009 formed the study cohort. We adopted a validated clinical score to quantitatively assess and document their neurological...
5 Citations Source Cite
Published on Jan 1, 2015in Current Opinion in Rheumatology 4.28
Zachary S. Wallace17
Estimated H-index: 17
 , John H. Stone65
Estimated H-index: 65
13 Citations Source Cite
Published on Jun 1, 2014in JAMA Neurology 11.46
Lucy X. Lu1
Estimated H-index: 1
 (Vanderbilt University), Emanuel Della-Torre13
Estimated H-index: 13
 (Vita-Salute San Raffaele University)+ 1 AuthorsStephen W. Clark5
Estimated H-index: 5
 (Vanderbilt University)
Importance IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is an increasingly recognized manifestation of IgG4-related disease, a fibroinflammatory condition that can affect virtually any organ. It is estimated that IgG4-RHP may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. Objective To summarize the current knowledge on IgG4-RHP including its pathological, clinical, and radiological presentations. Particular emphasis is placed on diagnos...
75 Citations Source Cite
Published on Mar 3, 2016in Scandinavian Journal of Rheumatology 3.02
Corrado Campochiaro10
Estimated H-index: 10
 , Ga Ramirez1
Estimated H-index: 1
 + 14 AuthorsMoreno Tresoldi9
Estimated H-index: 9
Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as ‘definite’ or ‘possible’ according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means o...
34 Citations Source Cite
Published on Jun 1, 2016in Practical Neurology
Thomas Williams2
Estimated H-index: 2
 (Queen Mary University of London), Monica Marta18
Estimated H-index: 18
 (Queen Mary University of London), Gavin Giovannoni69
Estimated H-index: 69
 (Queen Mary University of London)
Hypertrophic pachymeningitis secondary to IgG4-related disease is a rare but sometimes devastating cause of intracranial hypertension. It has the potential for an excellent response to corticosteroids or rituximab. We discuss the clinical presentation, imaging, histology (with its difficult distinction from lymphoma), management and follow-up of a case, including relapse and re-treatment following an initial response to rituximab.
3 Citations Source Cite
Published on Apr 1, 2016in The Journal of Rheumatology 3.47
Sowmini Medavaram (Newark Beth Israel Medical Center), Feifei Xue (Newark Beth Israel Medical Center), Robert G. Lahita3
Estimated H-index: 3
 (Newark Beth Israel Medical Center)
To the Editor: Immunoglobulin G4-related disease (IgG4-RD) is a recognized systemic condition that can affect almost every organ system1. The most commonly involved organs are the pancreas, salivary glands, and biliary tree. The common presentation with central nervous system (CNS) involvement is hypophysitis and pachymeningitis, but rarely leptomeningitis2. Only 3 reported cases of leptomeningitis have been reported, all of them presenting as cognitive decline, and 2 associated with rheumatoid ...
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