Ocular involvement in idiopathic hypertrophic pachymeningitis associated with
            eosinophilic angiocentric fibrosis: a case report

Suárez, Antonio Ramos; Capote, Ana Chinchurreta; Barrientos, Yolanda; Soto, Mercedes Lorenzo; Moreno, Saturnino Gismero; Martín, Fernando García

doi:10.5935/0004-2749.20180050

Cited by 7 publications

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“…Three steroid-refractory HP patients treated with RTX for 4 weeks showed clinical improvements and exhibited prominent decreases in dural thickness (61). Thus, RTX has been suggested to be a second-line therapy for steroid-refractory HP, especially for IgG4-RD (52)(53)(54)(55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65).…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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Aims: To investigate the causes, clinical characteristics, imaging features, and therapeutic implications of hypertrophic pachymeningitis (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, and neuroimaging results 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 antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). Headache was the most common symptom. The most frequently changed laboratory finding was elevated erythrocyte sedimentation rate (ESR). Imaging was characterized by cerebral or spinal dura mater 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 posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment was applied in most cases. Conclusions: The etiology of HP varied among patients, with idiopathic HP being the most common. MRI showed enhancement of the dura mater, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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“…Three steroid-refractory HP patients treated with RTX for four weeks showed clinical improvements and exhibited prominent decreases in dural thickness [55]. Thus, RTX has been suggested to be a second-line therapy for steroid-refractory HP, especially for IgG4-RD [56][57][58][59][60].…”

Section: Treatmentmentioning

confidence: 99%

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Xiao

Feng

2020

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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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“…Of the remaining seven cases, a further five cases 2,3,7,11,20 were noted to lack sufficient data, including serum IgG4 levels and/or IHC investigations for IgG4. One case 16 did not meet the classification criteria despite furnishing complete data. However, the patient in this case concurrently had idiopathic hypertrophic pachymeningitis – another clinical entity associated with IgG4‐RD.…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

et al. 2022

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Eosinophilic angiocentric fibrosis (EAF) has been postulated to lie on the spectrum of IgG4‐related disease (IgG4‐RD) in a study reported in 2011. However, the aetiology of EAF remains controversial, despite increasing literature reporting IgG4‐RD targeted investigations in those studies. This review aims to formally evaluate the relationship between EAF and IgG4‐RD using the latest 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification scheme for IgG4‐RD. A literature search was performed on the PubMed database for all studies describing patients diagnosed with EAF. Additionally, two EAF cases were retrospectively identified from our institution and presented. The demographics, clinical presentation, histological and serological data of each patient were analysed. A total of 20 cases were evaluated, including 18 patients from 14 articles in the English literature and two cases from our institution. Six of 20 (30%) patients with EAF met the criteria for IgG4‐RD with the 2019 ACR/EULAR classification scheme. This represents the first attempt at formally evaluating the relationship between EAF and IgG4‐RD using a validated set of classification criteria. Using the currently available data, a small proportion of patients with EAF met the criteria for IgG4‐RD. However, various limitations of this study suggest that further efforts at disease characterization of EAF may be required.

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Ocular involvement in idiopathic hypertrophic pachymeningitis associated with eosinophilic angiocentric fibrosis: a case report

Cited by 7 publications

References 0 publications

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

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