Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

Shimojima, Yasuhiro; Kishida, Dai; Ichikawa, Takanori; Kida, Takashi; Yajima, Nobuyuki; Omura, Satoshi; Nakagomi, Daiki; Abe, Yoshiyuki; Kadoya, Masatoshi; Takizawa, Naoho; Nomura, Atsushi; Kukida, Yuji; Kondo, Naoya; Yamano, Yasuhiko; Yanagida, Takuya; Endo, Koji; Hirata, Shintaro; Matsui, Kiyoshi; Takeuchi, Tohru; Ichinose, Kunihiro; Kato, Masaru; Yanai, Ryo; Matsuo, Yusuke; Nishioka, Ryo; Okazaki, Ryota; Takata, Tomoaki; Ito, Takafumi; Moriyama, Mayuko; Takatani, Ayuko; Miyawaki, Yoshia; Ito-Ihara, Toshiko; Kawaguchi, Takashi; Kawahito, Yutaka; Sekijima, Yoshiki

doi:10.1186/s13075-022-02898-4

Table 2 Incidence and scores of manifestations related to AAV between AAV patients with and without HP

From: Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

	Total AAV			Newly diagnosed AAV			Relapsed AAV
	HP (n = 30)	Non-HP (n = 633)	p-values	HP (n = 20)	Non-HP (n = 538)	p-values	HP (n = 10)	Non-HP (n = 95)	p-values
BVAS, total
Total score	13.4 ± 5.56	14.9 ± 7.11	0.364	14.0 ± 5.40	15.7 ± 7.01	0.362	12.3 ± 5.98	10.6 ± 6.02	0.390
Manifestation
Incidence, number (%)
General	13 (43)	367 (58)	0.132	11 (55)	335 (62)	0.640	2 (20)	32 (34)	0.494
Cutaneous	2 (7)	149 (24)	0.042	2 (10)	136 (25)	0.184	0	13 (14)	0.357
Mucous membranes/eyes	11 (37)	55 (8)	< 0.0001	6 (30)	47 (9)	0.007	5 (50)	8 (8)	0.002
ENT	16 (53)	157 (25)	0.001	13 (65)	135 (25)	0.0002	3 (30)	22 (23)	0.698
Chest	10 (33)	272 (46)	0.194	7 (35)	255 (47)	0.363	3 (30)	35 (37)	0.745
Cardiovascular	0	46 (7)	0.257	0	42 (8)	0.388	0	4 (4)	0.999
Abdominal	0	24 (4)	0.619	0	21 (4)	0.999	0	3 (3)	0.999
Renal	12 (40)	427 (67)	0.003	10 (50)	381 (71)	0.077	2 (20)	46 (48)	0.106
Nervous system	25 (83)	232 (37)	< 0.0001	18 (90)	195 (36)	< 0.0001	7 (70)	37 (39)	0.090
Score^a
General	1.00 ± 1.25	1.34 ± 1.28	0.153	1.35 ± 1.31	1.47 ± 1.28	0.612	0.40 ± 0.84	0.63 ± 0.99	0.421
Cutaneous	0.13 ± 0.51	0.58 ± 1.17	0.029	0.20 ± 0.62	0.63 ± 1.21	0.111	0	0.29 ± 0.89	0.215
Mucous membranes/eyes	1.62 ± 2.40	0.30 ± 1.13	< 0.0001	1.05 ± 1.93	0.29 ± 1.07	0.013	2.60 ± 2.99	0.40 ± 1.42	0.0001
ENT	2.52 ± 2.62	0.95 ± 1.88	< 0.0001	3.05 ± 2.56	0.95 ± 1.87	< 0.0001	1.50 ± 2.55	0.94 ± 1.96	0.529
Chest	1.50 ± 2.29	2.07 ± 2.41	0.184	1.45 ± 2.16	2.13 ± 2.41	0.191	1.60 ± 2.63	1.69 ± 2.38	0.834
Cardiovascular	0	0.40 ± 1.46	0.126	0	0.43 ± 1.52	0.194	0	0.22 ± 1.09	0.510
Abdominal	0	0.30 ± 1.58	0.278	0	0.32 ± 1.63	0.368	0	0.22 ± 1.25	0.571
Renal	3.13 ± 4.45	6.37 ± 5.10	0.0008	3.60 ± 4.38	6.85 ± 5.07	0.005	2.20 ± 4.66	3.66 ± 4.46	0.191
Nervous system	3.53 ± 3.22	2.61 ± 3.73	0.0008	3.30 ± 3.16	2.64 ± 3.76	0.002	4.00 ± 3.46	2.48 ± 3.54	0.114

Statistical significance was set at a p-value < 0.05. Values are expressed as mean ± SD
ANCA antineutrophil cytoplasmic antibody, AAV ANCA-associated vasculitis, BVAS Birmingham Vasculitis Activity Score version 3, ENT eye, nose, and throat, HP hypertrophic pachymeningitis
^aScored for each manifestation item based on the BVAS [21]

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