Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis

Della‐Torre, Emanuel; Galli, Laura; Franciotta, Diego; Bozzolo, Enrica; Briani, Chiara; Furlan, Roberto; Roveri, Luisa; Sessa, Maria; Passerini, Gabriella; Sabbadini, Maria Grazia

doi:10.1016/j.jneuroim.2013.10.008

Cited by 64 publications

(47 citation statements)

References 15 publications

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“…Similar to ANCA related HP, serum levels of IgG4 has not reliably predicted the presence of IgG4+ plasma cells on biopsy of dura or other organ systems [10,11]. CSF IgG4 may be more predictive [12], but this was not assessed in our set of cases.…”

Section: Distribution Of Cases and Demographicsmentioning

confidence: 71%

Hypertrophic pachymeningitis

Hahn

Fulbright

Baehring

2016

Journal of the Neurological Sciences

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Hypertrophic Pachymeningitis (HP) denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. Clinically, HP can present as debilitating headaches and cranial nerve defects but in other cases may be completely asymptomatic. We aimed to determine the relative incidence of different etiologies of HP and compare their associated imaging findings. Additionally, we sought to compare the clinical features of the underlying syndromes. We retrospectively examined twenty-two consecutive cases of HP seen in a single practitioner neurology practice over a ten-year time period. The most common etiologies were idiopathic HP and neurosarcoidosis. No imaging features were completely specific to any etiology. Nonetheless, idiopathic HP typically demonstrated diffuse regular enhancement whereas neurosarcoidosis was more likely to display a nodular enhancement pattern. Headache and cranial neuropathies were the most common clinical presentation. HP symptoms were often responsive to steroids but complete responses were rare. HP is a diagnostic challenge without specific findings on minimally or non-invasive diagnostic studies. Biopsy is often required and serves as the basis for effective therapy.

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Section: Distribution Of Cases and Demographicsmentioning

confidence: 71%

Hypertrophic pachymeningitis

Hahn

Fulbright

Baehring

2016

Journal of the Neurological Sciences

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“…IgG4-RD was originally described in the pancreas as type 1 autoimmune pancreatitis (AIP) (1,2), and subsequently reported in every organ system owing to the recognition of common histopathological features (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). Indeed, histopathological examination is the current gold standard for the diagnosis of IgG4-RD because clinical manifestations as well as serological findings are largely non-specific, and overlap with various inflammatory and neoplastic conditions (11)(12)(13).…”

mentioning

confidence: 99%

IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients

Campochiaro

Ramirez

Bozzolo

et al. 2015

Scandinavian Journal of Rheumatology

123

125

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“…A raised serum IgG4 concentration adds further support to the diagnosis of systemic IgG4-RD,10 though this is less sensitive for disease confined to the meninges 11. CSF analyses, however, appear more promising—intrathecal IgG4 production in IgG4-HP leads to oligoclonal bands and raised CSF IgG4 that can reliably distinguish cases from controls and alternative causes of hypertrophic pachymeningitis 11…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension

2015

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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.

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Diagnostic value of IgG4 Indices in IgG4-Related Hypertrophic Pachymeningitis

Cited by 64 publications

References 15 publications

Hypertrophic pachymeningitis

Hypertrophic pachymeningitis

IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients

IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension

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