Journal of neuroimmunology · 2026
Clinical characteristics of IgG4-related hypertrophic pachymeningitis with and without serum ANCA positivity.
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Zhuang Qingqing, Quan Chao, Zhangbao Jingzi, Gao Mingshi, Tan Haibo, Mao Yiting, Li Xiang
Аннотация
BACKGROUND: IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is a rare immune-mediated fibroinflammatory disorder. Some patients show concurrent serum anti-neutrophil cytoplasmic antibody (ANCA) positivity, which may create diagnostic uncertainty with ANCA-associated vasculitis. We aimed to compare the clinical features of IgG4-RHP patients with and without serum ANCA positivity.
METHODS: We retrospectively collected pathologically confirmed IgG4-RHP cases from Huashan Hospital between January 2020 and April 2026. We also searched PubMed and Chinese core journals for biopsy-confirmed IgG4-RHP cases published between January 2014 and April 2026. Patients were divided into ANCA-positive and ANCA-negative groups for comparative analysis. For ANCA-positive cases, we additionally reviewed ANCA pattern/specificity and performed a criteria-informed descriptive phenotypic assessment.
RESULTS: A total of 67 patients with pathologically confirmed IgG4-RHP were included, including 4 patients from our center and 63 from the literature. Thirteen patients (19.4%) were serum ANCA-positive. Compared with ANCA-negative patients, ANCA-positive patients were more likely to present with headache (100.0% vs. 60.8%, p = 0.02) and diffuse dural thickening on MRI (100.0% vs. 51.0%, p = 0.001). Among the 13 ANCA-positive cases, 8 were descriptively considered more suggestive of an MPA-like phenotype and 5 of a GPA-like phenotype, while no case showed convincing EGPA-like features. No significant differences were observed in age at onset, sex, cranial nerve involvement, extracranial organ involvement, laboratory findings, or treatment patterns.
CONCLUSIONS: Serum ANCA positivity does not exclude the diagnosis of IgG4-RHP. ANCA-positive IgG4-RHP may represent a distinct clinicoradiological phenotype characterized by more frequent headache and diffuse dural involvement. Histopathological confirmation remains crucial for diagnosis.
METHODS: We retrospectively collected pathologically confirmed IgG4-RHP cases from Huashan Hospital between January 2020 and April 2026. We also searched PubMed and Chinese core journals for biopsy-confirmed IgG4-RHP cases published between January 2014 and April 2026. Patients were divided into ANCA-positive and ANCA-negative groups for comparative analysis. For ANCA-positive cases, we additionally reviewed ANCA pattern/specificity and performed a criteria-informed descriptive phenotypic assessment.
RESULTS: A total of 67 patients with pathologically confirmed IgG4-RHP were included, including 4 patients from our center and 63 from the literature. Thirteen patients (19.4%) were serum ANCA-positive. Compared with ANCA-negative patients, ANCA-positive patients were more likely to present with headache (100.0% vs. 60.8%, p = 0.02) and diffuse dural thickening on MRI (100.0% vs. 51.0%, p = 0.001). Among the 13 ANCA-positive cases, 8 were descriptively considered more suggestive of an MPA-like phenotype and 5 of a GPA-like phenotype, while no case showed convincing EGPA-like features. No significant differences were observed in age at onset, sex, cranial nerve involvement, extracranial organ involvement, laboratory findings, or treatment patterns.
CONCLUSIONS: Serum ANCA positivity does not exclude the diagnosis of IgG4-RHP. ANCA-positive IgG4-RHP may represent a distinct clinicoradiological phenotype characterized by more frequent headache and diffuse dural involvement. Histopathological confirmation remains crucial for diagnosis.