Pos0822 hypertrophic pachymeningitis in antineutrophil cytoplasmic antibody-associated vasculitis: a multicenter survey in japan

Background Hypertrophic pachymeningitis (HP), characterized by an inflammatory disorder indicating intracranial or spinal thickening of dura mater, is found to develop as a neurological involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Meanwhile, the previous studies focusing on HP in AAV have been reported as a single-institution study, and the analyses were performed in a small number of patients because HP is a rare neurological disorder. Therefore, neither etiological nor clinical characteristics of HP in AAV have been adequately elucidated. Objectives This study clarified the characteristics of HP in AAV by analyzing the information of multicenter study in Japan (Japan collaborative registry of ANCA-associated vasculitis: J-CANVAS). Methods We analyzed the clinical information from 541 Asian patients with AAV enrolled in J-CANVAS. Of them, newly diagnosed and relapsed AAV were included in 448 and 93, respectively. The epidemiological and clinical findings were compared between patients with and without HP. Clinical manifestations related to AAV were evaluated based on the Birmingham Vasculitis Activity Score version 3. To elucidate independent factors in HP development, logistic regression analyses were additionally performed. Results Of the total 541 patients (mean age: 71±14 years, M:F = 1:1.2), HP was demonstrated in 28 (5.17%), including 17 (3.79%) in newly diagnosed AAV and 11 (11.8%) in relapsed AAV. The classification of granulomatosis with polyangiitis (GPA) was significantly higher in patients with HP than those without HP (50% vs. 21%, p = 0.0007). In newly diagnosed AAV, patients with HP significantly had higher GPA classification and higher positivity for PR3-ANCA than those without HP (53% vs. 17%, p = 0.001; 29% vs. 9%, p = 0.015, respectively). Conversely, positivity for MPO-ANCA was significantly higher in patients with HP than those without HP in relapsed AAV (91% vs. 55%, p = 0.025), despite not significantly different in the classification of AAV. Headache and cranial neuropathies were significant neurological symptoms in patients with HP compared to those without HP (82% vs. 6.6%, p < 0.0001; 32% vs. 2.9%, p < 0.0001, respectively). Besides, ear, nose and throat (ENT) and mucous membranes/eyes were significantly higher involvements in patients with HP than in those without HP (54% vs. 26%, p = 0.003; 29% vs. 9%, p = 0.003, respectively). Moreover, higher complications of “conjunctive hearing loss” and “sudden visual loss”, which are included in the categories of ENT and mucous membranes/eyes involvement, respectively, were significantly indicated in patients with HP than those without HP (39% vs. 7.2%, p < 0.0001; 21% vs. 1.2%, p < 0.0001, respectively). Multivariable logistic regression analysis identified that ENT (odds ratio [OR] 1.28, 95% confident interval [CI] 1.09 to 1.49, p = 0.002) and mucous membranes/eyes involvement (OR 1.37, CI 1.14 to 1.65, p = 0.0006), as well as conjunctive hearing loss (OR 4.52, CI 1.56 to 13.05, p = 0.005) and sudden visual loss (OR 1.84, CI 1.12 to 3.00, p = 0.015), were independent related factors in patients with HP. Conclusion GPA could be significantly classified in patients with HP. Notably, patients with HP significantly showed higher positivity for PR3-ANCA than those without HP in newly diagnosed AAV. Furthermore, sudden visual loss and conjunctive hearing loss might be implicated in HP development. Disclosure of Interests None declared