Hypertrophic Cranial Pachymeningitis in Mpo-Anca-Related Vasculitis: A Case Report and Literature Review

Watanabe, Kimio; Tani, Yasuhiro; Kimura, Hiroshi; Asai, Jun; Tanaka, Kenichi; Hayashi, Yoshimitsu; Asahi, Koichi; Nakayama, Masaaki; Watanabe, Tsuyoshi

doi:10.5387/fms.59.56

Cited by 9 publications

(10 citation statements)

References 20 publications

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“…In fact, the prevalence of HP was significantly lower in newly diagnosed AAV than in relapsed AAV, supporting the theory that CNS involvement could develop in the chronic phase of AAV [ 6 , 7 ]. However, some cases of HP in AAV, whose initial diagnosis of MPA was changed to GPA, have been reported [ 8 , 19 , 25 ], suggesting that HP could be the initial episode of GPA.…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

et al. 2022

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Background This study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan. Methods We analyzed the clinical information of 663 Asian patients with AAV (total AAV), including 558 patients with newly diagnosed AAV and 105 with relapsed AAV. Clinical findings were compared between patients with and without HP. To elucidate the relevant manifestations for HP development, multivariable logistic regression analyses were additionally performed. Results Of the patients with AAV (mean age, 70.2 ± 13.5 years), HP was noted in 30 (4.52%), including 20 (3.58%) with newly diagnosed AAV and 10 (9.52%) with relapsed AAV. Granulomatosis with polyangiitis (GPA) was classified in 50% of patients with HP. A higher prevalence of GPA was significantly observed in patients with HP than in those without HP in total AAV and newly diagnosed AAV (p < 0.001). In newly diagnosed AAV, serum proteinase 3 (PR3)-ANCA positivity was significantly higher in patients with HP than in those without HP (p = 0.030). Patients with HP significantly had ear, nose, and throat (ENT) (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.03–2.14, p = 0.033) and mucous membrane/eye manifestations (OR 5.99, 95% CI 2.59–13.86, p < 0.0001) in total AAV. Moreover, they significantly had conductive hearing loss (OR 11.6, 95% CI 4.51–29.57, p < 0.0001) and sudden visual loss (OR 20.9, 95% CI 5.24–85.03, p < 0.0001). Conclusion GPA was predominantly observed in patients with HP. Furthermore, in newly diagnosed AAV, patients with HP showed significantly higher PR3-ANCA positivity than those without HP. The ear and eye manifestations may be implicated in HP development.

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Section: Discussionmentioning

confidence: 99%

Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

et al. 2022

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“…It is notable that posterior fossa HP can cause acute noncommunicating hydrocephalus (9). In addition to the posterior fossa, thickened falx cerebri (10)(11)(12)(13)(14), anterior cranial fossa (15), cerebellar tentorium (10,16), frontal lobes (10,11,17), sphenoid wings (18), cavernous sinuses (19), and paranasal maxillary sinuses (19,20) were also involved in HP patients. With enhancements in the cerebral falx and tentorium cerebelli in coronal scanning, a typical "Eiffel Tower" (21,22) or "Benz" sign emerged (23).…”

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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“…It is notable that posterior fossa HP can cause acute noncommunicating hydrocephalus [9]. In addition to the posterior fossa, thickened falx cerebri [10][11][12][13][14], anterior cranial fossa [15], cerebellar tentorium [10,16], frontal lobes [10,11,17], sphenoid wings [18], cavernous sinuses [19], and paranasal maxillary sinuses [19,20] were also involved in HP patients.…”

Section: Imaging Featuresmentioning

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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Hypertrophic Cranial Pachymeningitis in Mpo-Anca-Related Vasculitis: A Case Report and Literature Review

Cited by 9 publications

References 20 publications

Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

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