EULAR Abstract Archive

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POS0822 (2022)

HYPERTROPHIC PACHYMENINGITIS IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS: A MULTICENTER SURVEY IN JAPAN

Y. Shimojima¹, D. Kishida¹, T. Ichikawa¹, T. Kida², N. Yajima³, S. Omura⁴, D. Nakagomi⁵, Y. Abe⁶, K. Masatoshi⁷, N. Takizawa⁸, A. Nomura⁹, Y. Kukida¹⁰, N. Kondo¹¹, Y. Yasuhiko¹², T. Yanagida¹³, K. Endo¹⁴, S. Hirata¹⁵, K. Kawahata¹⁶, K. Matsui¹⁷, T. Takeuchi¹⁸, K. Ichinose¹⁹, M. Kato²⁰, R. Yanai³, Y. Matsuo^21,22, A. Yamasaki²³, R. Nishioka²⁴, T. Takata²⁵, M. Moriyama²⁶, A. Takatani²⁷, T. Ito²⁸, Y. Miyawaki²⁹, T. Ito-Ihara³⁰, T. Kawaguchi³¹, Y. Kawahito⁴, Y. Sekijima³²

¹Shinshu University School of Medicine, Department of Medicine (Neurology and Rheumatology), Matsumoto, Japan
²Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Inflammation and Immunology, Kyoto, Japan
³Showa University School of Medicine, Division of Rheumatology, Department of Medicine, Tokyo, Japan
⁴Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Inflammation and Immunology, Kyoto, Japan
⁵University of Yamanashi Hospital, Department of Rheumatology,, Chuo-shi, Japan
⁶Juntendo University, Department of Internal Medicine and Rheumatology, Tokyo, Japan
⁷Japanese Red Cross Society Kyoto Daiichi Hospital, Center for Rheumatic Disease, Kyoto, Japan
⁸Chubu Rosai Hospital, Department of Rheumatology, Nagoya, Japan
⁹St. Luke’s International Hospital, Immuno-Rheumatology Center, Tokyo, Japan
¹⁰Japanese Red Cross Society Kyoto Daini Hospital, Department of Rheumatology, Kyoto, Japan
¹¹Kyoto Katsura Hospital, Department of Nephrology,, Kyoto, Japan
¹²Tosei General Hospital, Department of Respiratory Medicine and Allergy, Seto, Japan
¹³Kagoshima University Hospital, Department of Hematology and Rheumatology, Kagoshima, Japan
¹⁴Tottori Prefectural Central Hospital, Department of General internal medicine, Tottori, Japan
¹⁵Hiroshima University Hospital, Department of Clinical Immunology and Rheumatology, Hiroshima,
¹⁶St. Marianna University School of Medicine, Division of Rheumatology and Allergy, Department of Internal Medicine, Kawasaki, Japan
¹⁷Hyogo College of Medicine, Department of Diabetes, Endocrinology and Clinical Immunology, Nishinomiya, Japan
¹⁸Osaka Medical and Pharmaceutical University, Department of Internal Medicine (IV), Takatsuki, Japan
¹⁹Nagasaki University Graduate School of Biomedical, Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences,, Nagasaki, Japan
²⁰Graduate School of Medicine, Hokkaido University, Department of Rheumatology, Endocrinology and Nephrology, Sapporo, Japan
²¹Tokyo Kyosai Hospital, Department of Rheumatology, Tokyo, Japan
²²Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Department of Rheumatology, Tokyo, Japan
²³Faculty of Medicine, Tottori University, Division of Respiratory Medicine and Rheumatology, Department of Multidisciplinary Internal Medicine, Yonago, Japan
²⁴Graduate School of Medical Science, Kanazawa University, Department of Rheumatology, Kanazawa, Japan
²⁵Tottori University, Division of Gastroenterology and Nephrology, Yonago, Japan
²⁶Shimane University Faculty of Medicine, Department of Rheumatology, Izumo, Japan
²⁷Sasebo Chuo Hospital, Rheumatic Disease Center, Sasebho, Japan
²⁸Shimane University Hospital, Division of Nephrology, Izumo, Japan
²⁹Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama, Japan
³⁰University Hospital, Kyoto Prefectural University of Medicine, The Clinical and Translational Research Center, Kyoto, Japan
³¹Tokyo University of Pharmacy and Life Sciences, Department of Practical Pharmacy, Tokyo, Japan
¹Shinshu University School of Medicine, Department of Medicine (Neurology and Rheumatology), Matsumoto, 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

Citation: , volume 81, supplement 1, year 2022, page 702

Session: Vasculitis — small vessel vasculitis (POSTERS only)

version:	1.02