A nationwide survey of hypertrophic pachymeningitis in Japan

Yonekawa, Tomomi; Murai, Hiroyuki; Utsuki, Satoshi; Matsushita, Takuya; Kitajima, Masaki; Isobe, Noriko; Yamasaki, Ryo; Yoshida, Mari; Kusunoki, Susumu; Sakata, Kiyomi; Fujii, Kiyotaka; Kira, Jun Ichi

doi:10.1136/jnnp-2013-306410

Cited by 155 publications

(186 citation statements)

References 26 publications

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“…The present study, together with previous descriptions, 2,3 suggests that HP may occur as the first involvement in approximately half of patients with AAVrelated HP who frequently present with headache and/ or cranial neuropathy as a common symptom, in addition to ENT involvement. In this regard, we should consider the previous description that the disease severity eventually developed into a systemic type of AAV in 18% of patients with MPO-ANCA positivity and 50% of those with PR3-ANCA positivity, even though they had been categorized as 'CNS-limited type' (i.e., HP without other visceral disorders), at the disease onset.…”

Section: Discussionmentioning

confidence: 86%

“…It is realized that intracranial infections, neoplastic diseases and some autoimmune disorders such as sarcoidosis and immunoglobulin G4 (IgG4)-related disease contribute to the development of HP, although cases of 'idiopathic HP' where etiology is uncertain still exist. 1,2 Considering the etiology of HP, which is associated with autoimmune disorders, anti-neutrophil cytoplasmic antibody (ANCA)-related HP is the most frequently recognized. 2,3 ANCA is implicated in small vessel vasculitis, which is designated as ANCA-associated vasculitis (AAV), and is distinct from immune complexmediated vasculitis.…”

Section: Introductionmentioning

confidence: 99%

“…1,2 Considering the etiology of HP, which is associated with autoimmune disorders, anti-neutrophil cytoplasmic antibody (ANCA)-related HP is the most frequently recognized. 2,3 ANCA is implicated in small vessel vasculitis, which is designated as ANCA-associated vasculitis (AAV), and is distinct from immune complexmediated vasculitis. 4 AAV is categorized as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) based on the phenotypes of ANCA, the histopathological findings of granulomatosis and/or necrotizing vasculitis formation, and clinical findings such as prior asthma, existence of respiratory tract lesions, and/or urinary tract involvement.…”

Section: Introductionmentioning

confidence: 99%

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Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

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Aim: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods:We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.Results: Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.Conclusion: AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.

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Section: Discussionmentioning

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

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“…Demographically, the median age of patients with idiopathic hypertrophic pachymeningitis is 58.3 years (standard deviation, 15.8; range, 37-88 years). [4][5][6] Only a few paediatric patients have been reported, with the youngest age being 2 years and 11 months in India. 7 Our patient had early-onset disease.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

Lok¹,

Yip²,

Chong³

et al. 2015

Hong Kong Med J

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A B S T R A C TIdiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. We report on a 28-year-old Chinese woman with a history of granulomatous mastitis 7 years previously and oligomenorrhoea, headache, blurred vision, and raised prolactin level 2 years previously, that was diagnosed as prolactinoma and treated conservatively with bromocriptine. However, she had recurrent bilateral vision loss when the bromocriptine was stopped. Her symptoms were resolved by high-dose steroid injection but remained steroid-dependent. Serial magnetic resonance imaging scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy. Lumbar puncture showed lymphocytosis with no organisms. Open biopsy of the meninges was performed and histology showed Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

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“…Cerebrospinal-fluid analysis (CSF) showed hyperproteinorachia (0.86 g/L) with normal glycorrhachia, 64 leukocytes/mm 3 with 93% activated non-clonal lymphocytes, mostly CD4+. A PCR assay to detect Mycobacterium tuberculosis DNA was negative.…”

Section: Mri Excluded Cerebral Venous Sinus Thrombosismentioning

confidence: 99%

Idiopathic hypertrophic pachymeningitis presenting with occipital neuralgia

Auboire¹,

Boutémy²,

Constans³

et al. 2015

Afr H. Sci.

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Abstract:Background: Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. Methods: We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Results and conclusions: Idiopathic hypertrophic pachymeningitis is a plausible cause of occipital neuralgia and may present without cranial-nerve palsy. There is no consensus on the treatment for idiopathic hypertrophic pachymeningitis, but the usual approach is to start corticotherapy and then to add immunosuppressants. When occipital neuralgia is not clinically isolated or when a first-line treatment fails, another disease diagnosis should be considered. However, the cost effectiveness of extended investigations needs to be considered.

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A nationwide survey of hypertrophic pachymeningitis in Japan

Abstract: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

Cited by 155 publications

References 26 publications

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

Idiopathic hypertrophic pachymeningitis presenting with occipital neuralgia

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