Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China

• Published in: Arthritis research & therapy Vol. 26; no. 1; pp. 6 - 10
• Main Authors: Chen, Yiran, Gou, Lijuan, Wang, Li, Qian, Min, Wu, Qingjun, Zheng, Wenjie, Li, Mengtao, Zeng, Xiaofeng, Zhang, Fengchun
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 02.01.2024; BioMed Central; BMC
• Subjects: Analysis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies; Antibodies, Antineutrophil Cytoplasmic; Antineutrophil cytoplasmic; Arthritis; Autoantibodies; Biopsy; Blindness - complications; Care and treatment; Complications and side effects; Diagnosis; Diagnostic tests; Granulomatosis with polyangiitis; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - drug therapy; Hearing loss; Hearing Loss, Sensorineural - complications; Humans; Hypertrophic cranial pachymeningitis; Induction therapy; Inflammatory diseases; Laboratories; Magnetic resonance imaging; Medical imaging; Medical records; Meningitis; Meningitis - complications; Nervous system; Neuroimaging; Neurologic manifestations; Neurologic manifestations of general diseases; Neurological disorders; Patients; Proteins; Remission (Medicine); Retrospective Studies; Risk factors; Testing; Vein & artery diseases; Wegener's granulomatosis; China; Antibodies; Granulomatosis with polyangiitis; Antineutrophil cytoplasmic; Meningitis; Neurologic manifestations; Hypertrophic cranial pachymeningitis
• ISSN: 1478-6362; 1478-6354; 1478-6362
• DOI: 10.1186/s13075-023-03239-9

Hypertrophic cranial pachymeningitis (HCP) is uncommon but a poorly understood complication of granulomatosis with polyangiitis (GPA). We conducted this retrospective study to elucidate the clinical characteristics and factors independently associated with granulomatosis with polyangiitis (GPA) complicated by hypertrophic cranial pachymeningitis (HCP) in China. We collected the medical records of 78 patients diagnosed with GPA who were admitted to the inpatient department of Peking Union Medical College Hospital between January 2003 and September 2021. Clinical features, laboratory and radiological findings, and Birmingham Vasculitis Activity Scores (excluding meningitis score) were recorded. A binary logistic regression analysis was performed to analyze factors independently associated with GPA-related HCP. Headache (100%) and cranial nerve palsy (61.5%) were common manifestations of HCP. Compared to 52 GPA patients without HCP, 26 patients with HCP required more time from initial symptoms to diagnosis, with a lower ratio of pulmonary and renal involvement, a higher ratio of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, conductive or sensorineural hearing loss, mastoiditis, and decreased vision or sudden visual loss. Binary logistic regression analysis indicated that proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) negativity (OR 10.698, p = 0.001), conductive or sensorineural hearing loss (OR 10.855, p = 0.005), and decreased vision or sudden visual loss (OR 8.647, p = 0.015) were significantly associated with GPA-related HCP. Of the 26 patients, 18 received methylprednisolone pulse treatment, and 18 received intrathecal injections of dexamethasone and methotrexate. HCP was a severe manifestation of GPA in our study. Independent factors associated with the occurrence of HCP in patients with GPA included PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased vision or sudden visual loss. Furthermore, GPA-related HCP was associated with higher disease activity, requiring more intensive treatments.