Hypertrophic pachymeningitis

Hahn, Lewis D.; Fulbright, Robert K.; Baehring, Joachim M.

doi:10.1016/j.jns.2016.06.024

Cited by 61 publications

(59 citation statements)

References 32 publications

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“…There have been reported significant overlaps in the distribution pattern of dural enhancement among patients with MPO–ANCA positive HP, PR3-ANCA positive HP, immune-mediated, and idiopathic HP, and there is no consensus on the correlation between dural enhancement pattern and HP etiology [27, 39]. But fortunately, recent imaging studies indicated that most patients with MPO–ANCA positive HP have the CNS-limited form and less frequent leptomeningeal or parenchymal involvement than those with PR3-ANCA-positivity [36, 37].…”

Section: Discussionmentioning

confidence: 99%

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

Peng

Yang

et al. 2018

Eur Arch Otorhinolaryngol

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Background and objectiveIt has been recognized that anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides may lead to hypertrophic pachymeningitis (HP) or intractable otitis media (OM). To our knowledge, few cases of coexistent ANCA-related HP and OM have been described previously. To increase awareness of this disease, we reviewed the literature describing patients with HP and intractable OM in a population with AAV to guide clinical decision making for otolaryngologists.Methods PubMed was searched with the following terms: ANCA-associated vasculitis, otitis media, and hypertrophic pachymeningitis. Only patients with concomitant AAV, OM and HP were considered and included in this review.ResultsA total of 243 articles were reviewed, and of these, 6 met inclusion criteria. Headache, cranial polyneuropathy, and intractable OM with effusion or granulation were common. Serum MPO–ANCA positivity was most common in Asian patients. Almost all patients had dural mater thickening on gadolinium-enhanced magnetic resonance imaging of the brain. Corticosteroids plus an immunosuppressant was more effective and most patients had improved hearing after treatment, but approximately 50% of subjects had disease relapse.ConclusionIn this review, we summarized the current knowledge on the clinical features, diagnosis, treatment, and pathogenesis of this disease. We should carefully detect the potential cases of ANCA-related HP and OM in patients with intractable OM, HP, or AAV, and make the optimal treatment plan to avoid long-term neurological complications and irreversible hearing loss. Furthermore, due to an increased possibility of relapse, close follow-up, including a hearing test, ANCA titers, imaging examination, and detection of toxic and side effects of immunosuppressive therapy, are necessary.

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Section: Discussionmentioning

confidence: 99%

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

Peng

Yang

et al. 2018

Eur Arch Otorhinolaryngol

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“…We collected clinical, laboratory, imaging, and therapeutic data from these patients from our electronic medical records system. MRI enhancements were categorized by their locations, distributions, and patterns according to a definition described by Hahn et al [6]. Locations were identified by an experienced radiologist.…”

Section: Methodsmentioning

confidence: 99%

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Xiao

Feng

2020

Preprint

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Background Hypertrophic pachymeningitis (HP) is a fibrotic disorder featuring a thickening of the dura matter. Most HP studies were from Caucasian population and only a few studies of HP are available in China. In this study, we investigated the causes, clinical and imaging features and therapeutic implications of HP in a southern Chinese population.Methods We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, neuroimaging results, and clinical course were evaluated in all HP patients.Results The mean age at onset was 50±12 years. The most common diagnosis was idiopathic HP (67%), followed by ANCA-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). The main clinical manifestations were headache and cranial nerve deficits. The most frequently changed laboratory finding was elevated ESR. Imaging was characterized by cerebral or spinal dura matter enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal and occipital lobes for ANCA-related HP; and the posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment were applied in most cases.Conclusions HP was diagnosed based on clinical manifestations, imaging and laboratory results. Etiology varied among patients, with idiopathic HP being the most common. MRI with contrast showed enhancement of the dura matter, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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“…Pachymeningitis secondary to IgG4-RD is a form of hypertrophic pachymeningitis characterised by localised or diffuse inflammation and thickening of the meninges (mostly dura mater)—cerebral, spinal or rarely both [15, 53–55]. Infectious, inflammatory and neoplastic causes need to be considered.…”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

“…Infectious, inflammatory and neoplastic causes need to be considered. Of the inflammatory causes, sarcoidosis, granulomatosis with polyangiitis (GPA), rheumatoid arthritis (RA), Sjögren’s syndrome and IgG4-RD are the most common [15, 54, 55]. The prevalence of hypertrophic pachymeningitis has been evaluated in a Japanese national survey and reported to be 0.949 cases per 100,000, with pachymeningitis due to IgG4-RD being observed in 8.8%, second only to anti-neutrophil cytoplasmic antibody (ANCA)-related pachymeningitis [56].…”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

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Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-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 IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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Hypertrophic pachymeningitis

Cited by 61 publications

References 32 publications

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Neurological Manifestations of IgG4-Related Disease

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