Auris Nasus Larynx 48 (2021) 885–889

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Auris Nasus Larynx

journal homepage: www.elsevier.com/locate/anl

The association between ear involvement and clinical features

and prognosis in ANCA-associated vasculitis
Yuki Hosokawa a,b, Masahiro Okada a,∗, Koichiro Suemori c, Naohiko Hamaguchi d,
Ken-ichi Miyoshi d, Taro Takagi a, Masato Teraoka a, Hiroyuki Yamada a,
Jun Ishizaki c, Takuya Matsumoto c, Naohito Hato a
a Department of Otolaryngology, Head and Neck Surgery, Ehime University Graduate School of Medicine, Toon, Japan
b Department of Otolaryngology, Head and Neck Surgery, Ehime Prefectural Central Hospital, Matsuyama, Japan
c Department of Hematology, Clinical Immunology and Infectious Disease, Ehime University Graduate School of Medicine, Toon, Japan
d Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, Toon, Japan

A R T I C L E I N F O A B S T R A C T

Article history: Objective: The concept of otitis media with ANCA-associated vasculitis (OMAAV) was recently
Received 19 October 2020 proposed by the study group of the Japan Otological Society. However, little is known about
Accepted 2 February 2021 the effect of ear involvement on the clinical features and prognosis of AAV. We investigate this
Available online 13 February 2021
issue in this study.

Keywords: Methods: We retrospectively examined 36 patients diagnosed with OMAAV and 44 patients
Otitis media diagnosed with AAV without ear involvement (non-OMAAV) at Ehime University Hospital from
ANCA-associated vasculitis 2013 to 2018. We collected serological findings including ANCA type and titer, C-reactive
Wegener’s granulomatosis protein (CRP), serum creatinine level, organ involved at initial diagnosis, treatment, remission,
Microscopic polyangiitis disease relapse, and mortality from medical records. We investigated whether clinical features
Churg-Strauss syndrome and outcomes differed between the OMAAV and non-OMAAV groups.
Results: Age, ANCA titer, and CRP at initial diagnosis were not significantly different between
the two groups, and the rate of intravenous cyclophosphamide (IVCY) use also did not differ.
The proportions of patients with concurrent eye involvement, facial palsy (FP), and hypertrophic
pachymeningitis (HCP) were significantly higher in the OMAAV than in the non-OMAAV group
(p = 0.005, 0.005 and 0.049, respectively), while both renal and peripheral nerve involvement
were significantly less common in OMAAV patients (p = 0.04). Among the 30 patients with
renal involvement, serum creatinine level at diagnosis was significantly lower in the OMAAV
group (p = 0.04). The mortality rate was 8.3% in OMAAV and 6.8% in non-OMAAV cases,
but this difference was not significant. The rate of relapse was 33.3% in OMAAV and 13.6% in
non-OMAAV cases; this difference was significant (p = 0.04).
Conclusions: Serological measurements of disease activity did not differ between the groups.
Eye involvement, FP, and HCP, however, were significantly more common in AAV with ear
involvement. In addition, renal involvement was less common and renal impairment was milder
in AAV with ear involvement. These findings can be considered clinical features. The relapse
rate was significantly higher in AAV with ear involvement.
© 2021 Oto-Rhino-Laryngological Society of Japan Inc. Published by Elsevier B.V. All rights
reserved.

∗ Corresponding author.
E-mail address: okadama@m.ehime-u.ac.jp (M. Okada).

https://doi.org/10.1016/j.anl.2021.01.023
0385-8146/© 2021 Oto-Rhino-Laryngological Society of Japan Inc. Published by Elsevier B.V. All rights reserved.
886 Y. Hosokawa, M. Okada, K. Suemori et al. / Auris Nasus Larynx 48 (2021) 885–889

1. Introduction Table 1. Comparison of demographics between OMAAV and non-OMAAV

groups.
Antineutrophil cytoplasmic antibodies (ANCA) to OMAAV non-OMAAV p value
myeloperoxidase (MPO) and proteinase 3 (PR3) are associ-
N 36 44
ated with primary vasculitis affecting small- to medium-sized Age (years) 68.0 ± 1.9 68.0 ± 2.1 1.00
vessels. This systemic vasculitis is known as ANCA- Sex (Female / Male) 27 / 9 24 / 20 0.06
associated vasculitis (AAV) [1,2]. The initial sign of AAV Follow-up period (months) 35.1 ± 2.7 36.4 ± 3.3 0.75
can be otological symptoms, but an early diagnosis of AAV Onset to diagnosis (months) 6.9 ± 2.2 3.8 ± 0.4 0.69
localized to the ear is difficult to make [3]. The study ANCA status
MPO-ANCA 27 (75.0 %) 34 (77.3 %) 0.81
group of the Japan Otological Society recently proposed the PR3-ANCA 5 (13.9 %) 0 (0 %) 0.01
concept of otitis media with AAV (OMAAV) [4–6]. It has ANCA-negative 4 (11.1 %) 9 (20.5 %) 0.26
been reported that the administration of immunosuppressants Double-positive 0 (0 %) 1 (2.3 %) 1.00
with glucocorticoids (GC) reduced disease relapse [4]. Our ANCA indicates antineutrophil cytoplasmic antibody; MPO, myeloperox-
previous studies showed that immune activity in patients with idase; OMAAV, otitis media with ANCA-associated vasculitis; PR3, pro-
OMAAV localized to the ear is equivalent to the activity teinase 3.
in patients with OMAAV who had disease involvement
of organs other than the ear [7], and six of 23 patients vasculitis following remission. In non-OMAAV cases, relapse
with OMAAV had a poor response to GC and intravenous was defined as the occurrence of new involvement or as the
cyclophosphamide (IVCY) and required administration of recurrence or worsening of the manifestation of clinical vas-
rituximab (RTX) [8]. These findings suggest that the progno- culitis following remission.
sis of OMAAV is similar to that of other types of AAV, yet The data were expressed as mean ± SEM. Differences
little is known about whether clinical features and outcomes in each parameter between groups were analyzed using the t
differ between OMAAV and other AAV diseases. Thus, we test, Pearson’s chi-square test, or Fisher’s exact test with JMP
investigated this issue in this study. for Macintosh (SAS Institute Inc., Cary, NC). Statistical tests
were based on a significance level of p < 0.05.
2. Materials and methods
3. Results
This retrospective study was approved by the institutional
review board (IRB) of Ehime University Medical Hospital Of 80 patients, 36 patients were diagnosed with OMAAV
(No. 1812007). Eighty patients diagnosed with AAV, includ- and 44 patients were diagnosed with AAV without ear in-
ing OMAAV, granulomatosis with polyangiitis (GPA), micro- volvement (non-OMAAV), including 30 patients with MPA,
scopic polyangiitis (MPA), and eosinophilic granulomatosis 2 patients with GPA, and 12 patients with EGPA. Of 36 cases
with polyangiitis (EGPA) at Ehime University Hospital from with OMAAV, 19 patients had MPA, 8 patients had GPA,
2013 to 2018 with a follow-up period of more than 1 year 1 patient had EGPA, and 8 patients had only ear involve-
were included in this study. Diagnosis of OMAAV was based ment. Table 1 shows the clinical data of OMAAV and non-
on the diagnostic criteria of OMAAV proposed by the Japan OMAAV patients. The average age was 68.0 ± 1.9 years in
Otological Society [4–6]. We collected the involved organs the OMAAV group and 68.0 ± 2.1 in the non-OMAAV group;
at diagnosis, serological findings including ANCA type and the difference was not significant (p = 1.00). Sex, follow-up
titer, CRP, serum level of creatinine at initial diagnosis, and period, and onset to diagnosis were not significantly differ-
remission, treatment, disease relapse, and mortality from med- ent between the two groups. The majority of patients were
ical records. We investigated whether each parameter differed MPO-ANCA positive; however, the ratio of PR3-ANCA was
between OMAAV and other AAV diseases (non-OMAAV). significantly higher in the OMAAV group (p = 0.01).
In this cohort, standard induction therapy involved the Table 2 shows a comparison of clinical features between
administration of GC (prednisolone [PSL] 0.5–1.0 mg/kg) the OMAAV and non-OMAAV groups. Eye involvement,
or intravenous administration of methylprednisolone (mPSL) hypertrophic pachymeningitis, and facial palsy were signif-
(1000 mg/day) for 3 days, tapered with PSL 0.5–1.0 mg/kg. icantly more common in OMAAV cases, while both kidney
Patients with severe pulmonary and renal disease or those who and peripheral nerve involvement were significantly less com-
had difficulty with the induction of remission using GC/mPSL mon in the OMAAV group. Serological markers of disease ac-
were treated with biweekly or monthly intravenous cyclophos- tivity including ANCA titer and CRP did not significantly dif-
phamide (500 mg) (IVCY) or plasma exchange. RTX was fer between the groups, though serum creatinine levels were
used for patients who had difficulty with the induction of significantly better in OMAAV compared to non-OMAAV
remission using mPSL and IVCY. cases at both diagnosis and remission (p = 0.003 and 0.01, re-
Remission was defined as a 3-month absence of manifes- spectively). Sixteen (44.4%) patients were treated with IVCY
tations attributable to AAV. In OMAAV cases, the definition in the OMAAV group and 11 (25.0%) in the non-OMAAV
of relapse was 1) the recurrence of otitis media or hearing group; the difference was not significant (p = 0.07). Ritux-
loss following remission and diseases other than AAV could imab was used in 12 (33.3%) OMAAV cases and in 4 (9.1%)
be excluded, 2) the occurrence of new involvement, and 3) non-OMAAV cases; the difference was significant (p = 0.01).
the recurrence or worsening of the manifestation of clinical Twelve (33.3%) OMAAV patients experienced disease re-
 Y. Hosokawa, M. Okada, K. Suemori et al. / Auris Nasus Larynx 48 (2021) 885–889 887

Table 2. Comparison of clinical features between OMAAV and non-OMAAV groups.

OMAAV non-OMAAV p value

Involvement at diagnosis: No. (%)
Eye 8 (22.2 %) 1 (2.3 %) 0.005
Nose 7 (19.4 %) 10 (22.7 %) 0.72
Lung (Interstitial lung disease) 16 (44.4 %) 18 (40.9 %) 0.75
Kidney 9 (25.0 %) 21 (47.7%) 0.04
Hypertrophic pachymeningitis 5 (13.9 %) 1 (2.3 %) 0.049
Facial palsy 6 (16.7 %) 0 (0 %) 0.005
Peripheral nerve 4 (11.1 %) 13 (29.5 %) 0.04
ANCA titer at diagnosis (U/mL) 119.3 ± 20.1 128.9 ± 17.0 0.71
CRP at diagnosis (mg/dL) 8.0 ± 1.2 5.7 ± 0.8 0.10
Serum creatinine (mg/dL) at diagnosis 0.9 ± 0.1 2.5 ± 0.4 0.003
at remission 0.9 ± 0.1 1.7 ± 0.3 0.01
Treatment: No. (%)
mPSL pulse 29 (80.6 %) 13 (29.5 %) <0.0001
IVCY 16 (44.4 %) 11 (25.0 %) 0.07
RTX 12 (33.3 %) 4 (9.1 %) 0.007
Relapse: No. (%) 12 (33.3 %) 6 (13.6 %) 0.04
Disease-related death: No. (%) 3 (8.3 %) 3 (6.8 %) 0.79
ANCA indicates antineutrophil cytoplasmic antibody; CRP, c-reactive protein; IVCY, intravenous
cyclophosphamide; mPSL, methylprednisolone; OMAAV, otitis media with ANCA-associated vas-
culitis; RTX, rituximab.

Table 3. Comparison of renal function among patients with kidney involvement.

OMAAV non-OMAAV p value

N 9 21
Age (years) 72.3 ± 2.1 70.2 ± 3.6 0.71
Sex (female / male) 5/4 9 / 12 0.52
Follow-up period (months) 31.3 ± 5.2 30.5 ± 4.2 0.91
CRP (mg/dL) 14.1 ± 3.3 4.2 ± 0.8 0.02
Serum creatinine (mg/dL) at diagnosis 1.6 ± 0.5 3.6 ± 0.7 0.03
at remission 1.3 ± 0.2 2.0 ± 0.3 0.15
CRP indicates c-reactive protein; OMAAV, otitis media with ANCA-associated vasculitis.

lapse, as did six (13.6%) non-OMAAV patients. The relapse
rate was significantly higher in OMAAV than in non-OMAAV
cases (p = 0.04). Disease-related and treatment-related mor-
tality were seen in three (8.3%) OMAAV patients and three
(6.8%) non-OMAAV patients. There was no significant dif-
ference in mortality rate between the groups (p = 0.79).
Among 30 patients with renal involvement (9 OMAAV
and 21 non-OMAAV), the serum level of creatinine at di-
agnosis was 1.6 ± 0.5 mg/dl in the OMAAV group and
3.6 ± 0.7 mg/dl in the non-OMAAV group, and the serum
level of creatinine was significantly lower in the OMAAV
group as well (p = 0.04) (Table 3).
4. Discussion
Although the number of reports of OMAAV is increasing
[4-14], little is known about the clinical features and prog-
nosis of OMAAV compared to other AAV diseases. In this
study, HCP and facial palsy were significantly more common
in OMAAV patients, and both kidney and peripheral nerve
involvement were less common in OMAAV cases. Renal im-
pairment was also significantly milder in the OMAAV group.
These outcomes can be considered to be the clinical features
of OMAAV. In addition, the relapse rate was significantly
higher in OMAAV cases although the use of IVCY, which is
a standard treatment for systemic AAV in Japan [15,16], was
equal to that for non-OMAAV cases.
The initial signs of AAV are reported to be otologic symp-
toms including facial palsy [3]. Together with facial palsy,
it has been suggested that HCP in AAV patients is related
to otitis media [4,17,18]. In a retrospective study of 235 pa-
tients with OMAAV, 28% of the patients had HCP throughout
their clinical course [4]. Another retrospective cohort study
reported that otitis media was the predictive factor of HCP in
AAV patients, and the odds ratio was 38.1 [17]. Thus, HCP
may be one of the clinical features of OMAAV. In cases of
intractable otitis media with HCP, clinicians should check the
ANCA status.
In this study, renal involvement was less common and re-
nal impairment was milder in the OMAAV group. It has been
reported that the presence of ENT involvement is associated
with a lower renal involvement rate [19,20] and mild renal
impairment [19–21]. Our results are consistent with those of
the previous reports. In terms of renal function, ear involve-
ment is related to favorable outcomes, yet the mortality rate
did not differ between the OMAAV and non-OMAAV groups.
In addition, OMAAV patients had a higher disease relapse
rate even though treatment intensity was similar between the
888 Y. Hosokawa, M. Okada, K. Suemori et al. / Auris Nasus Larynx 48 (2021) 885–889
groups. Harabuchi et al. reported that 41% of OMAAV pa-
tients experienced disease relapse [4], and this rate is similar
to that of our study (33.3% in the OMAAV group). Compared
to other prospective clinical trials of systemic AAV, which re-
ported a relapse rate of 15–20% [22–24], OMAAV patients
may have a higher disease relapse rate. The mechanism un-
derlying why AAV with ear involvement is related to a higher
relapse rate is unknown; however, it has been reported that
infection and the release of proinflammatory cytokines are
triggers of production for ANCA antibodies [25]. In addi-
tion, Morita et al. reported that MPO-DNA complex could
be detected in otitis media fluid in patients with OMAAV,
and levels of MPO-DNA complex and hearing threshold were
positively correlated [13], suggesting that middle ear infection
may trigger the presence of OMAAV. The middle ear cavity
is more easily exposed to pathogens than other organs are,
and OMAAV may have a higher relapse rate compared to
other types of AAV.
The rate of peripheral nerve involvement was significantly
lower in OMAAV. EGPA has a higher incidence of periph-
eral nerve involvement compared to other two AAV diseases
[26]. Since only one patient had EGPA in the OMAAV group
while 12 patients had EGPA in the non-OMAAV group, our
results reflected the difference in the proportion of patients
with EGPA between the two groups.
There are several limitations to this study. First, this study
was conducted in a single center, was retrospective, and the
number of patients was small. Large-scale prospective mul-
ticenter studies are therefore needed. Second, as initial ther-
apy differed among patients, there was the possibility of bias
in the relapse rate. However, the treatment intensity was the
same for both groups; the use of IVCY was equal, and the
use of mPSL pulse was even higher in OMAAV than in non-
OMAAV patients. Together with previous reports of a higher
relapse rate in OMAAV cases, it is suggested that OMAAV
patients had a higher relapse rate than did patients with other
types of AAV. Third, as the concept of OMAAV includes oti-
tis media caused by GPA, MPA and EGPA, as well as otitis
media that does not fulfill the ordinary diagnostic criteria for
systemic AAV [6], we investigated the differences in clini-
cal features and prognosis between OMAAV and AAV with-
out ear involvement, including MPA, GPA, and EGPA. Since
it has been reported that ear involvement is related to GPA
[27], however, it is better to investigate the clinical features
of OMAAV compared to only GPA without ear involvement.
This cohort included only two patients with GPA without ear
involvement, therefore, we could not investigate the compari-
son between OMAAV and GPA without ear involvement, Fur-
ther studies are needed to clarify this issue.
5. Conclusion
Serological measurements of disease activity did not differ
between OMAAV and non-OMAAV patients. Eye involve-
ment, FP, and HCP, however, were significantly more com-
mon in AAV with ear involvement cases and can be con-
sidered clinical features. The relapse rate was also higher in
AAV with ear involvement patients.
Disclosure statements
None declared.
Acknowledgements
The authors would like to thank the members of the
OMAAV study group in Japan.
References
[1] van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van
Es LA, et al. The autoantibodies against neutrophils and monocytes:
tool for diagnosis and marker of disease activity in Wegener’s granulo-
matosis. Lancet 1985;23:425–9.
[2] Jannette JC, Xiao H, Falk RJ. Pathogenesis of vascular inflamma-
tion by anti-neutrophil cytoplasmic antibodies. J Am Soc Nephrol
2006;17:1235–42.
[3] Wierzbicka M, Szyfter W, Puszczewicz M, Borucji L, Bartochowska A.
Otologic symptoms as initial manifestation of Wegener’s granulomato-
sis: diagnostic dilemma. Otol Neurotol 2011;32:996–1000.
[4] Harabuchi Y, Kishibe K, Tateyama K, Morita Y, Yoshida N, Kuni-
moto Y, et al. Clinical features and treatment outcomes of otitis media
with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis
(OMAAV): a retrospective analysis of 235 patients from a nationwide
survey in Japan. Mod Rheumatol 2017;27:87–94.
[5] Yoshida N, Iino Y. Pathogenesis and diagnosis of otitis media with
ANCA-associated vasculitis. Allergol Int 2014;63:523–32.
[6] Harabuchi Y., Kishibe K., Tateyama K., Morita Y., Yoshida N., Okada
M., et al. Clinical characteristics, the diagnostic criteria and manage-
ment recommendation of otitis media with antineutrophil cytoplasmic
antibody (ANCA)-associated vasculitis (OMAAV) proposed by Japan
Otological Society. Auris Nasus Larynx. in press.
[7] Okada M, Suemori K, Takagi D, Teraoka M, Yamada H, Hato N. Com-
parison of localized and systemic otitis media with ANCA-associated
vasculitis. Otol Neurotol 2017;38:e506–10.
[8] Okada M, Suemori K, Takagi D, Teraoka M, Yamada H, Ishizaki J,
et al. The treatment outcomes of rituximab for intractable otitis media
with ANCA-associated vasculitis. Auris Nasus Larynx 2019;46:38–42.
[9] Morita Y, Takahashi K, Izumi S, Kubota Y, Ohshima S, Horii A.
Vestibular involvement in patients with otitis media with an-
tineutrophil cytoplasmic antibody-associated vasculitis. Otol Neurotol
2017;38:97–101.
[10] Hisata Y, Sasaki E, Ishimaru K, Harada Y, Nakano H, Naitou S,
et al. Do you know otitis media with ANCA-associated vasculitis
(OMAAV)? J Gen Fam Med 2017;18:428–31.
[11] Kobari Y, Nagasawa T. Otitis media with ANCA-associated vasculitis:
a new concept and the associated criteria. Intern Med 2017;56:3365–7.
[12] Watanabe T, Yoshida H, Kishibe K, Morita Y, Yoshida N, Takahashi H,
et al. Cochlear implantation in patients with bilateral deafness caused
by otitis media with ANCA-associated vasculitis (OMAAV): a report of
four cases. Auris Nasus Larynx 2018;45:922–8.
[13] Morita S, Nakamaru Y, Nakazawa D, Suzuki M, Hoshino K, Fukuda A,
et al. The diagnostic and clinical utility of the myeloperoxidase-DNA
complex as a biomarker in otitis media with antineutrophil cytoplasmic
antibody-associated vasculitis. Otol Neurotol 2019;40:e99–e106.
[14] Morita Y., Kitazawa M., Yagi C., Nonomura Y., Takahashi K., Ya-
magishi T., et al. Tympanic membrane findings of otitis media
with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
(OMAAV). Auris Nasus Larynx. in press.
[15] Harigai M, Nagasaka K, Amano K, Bando M, Dobashi H, Kawakami T,
et al. Clinical practice guideline of the Japan research committee of
the ministry of health, labour, and welfare for intractable vasculitis
for the management of ANCA-associated vasculitis. Mod Rheumatol
2017;2019(29):20–30.
[16] Nagasaka K, Harigai M, Hagino N, Hara A, Horita T, Hayashi T,
et al. Systematic review and meta-analysis for 2017 clinical practice
 Y. Hosokawa, M. Okada, K. Suemori et al. / Auris Nasus Larynx 48 (2021) 885–889
guidelines of the japan research committee of the ministry of health,
labour, and welfare for intractable vasculitis for the management of
ANCA-associated vasculitis. Mod Rheumatol 2019;29:119–29.
[17] Imafuku A, Sawa N, Kawada M, Hiramatsu R, Hasegawa E, Ya-
manouchi M, et al. Incidence and risk factors of new-onset hypertrophic
pachymeningitis in patients with anti-neutrophil antibody-associated vas-
culitis: using logistic regression and classification tree analysis. Clin
Rheumatol 2019;38:1039–46.
[18] Peng A, Yang X, Wu W, Xiao Z, Xie D, Ge S. Anti-neutrophil
cytoplasmic antibody-associated hypertrophic cranial pachymeningitis
and otitis media: a review of literature. Eur Arch Otorhinolaryngol
2018;275:2915–23.
[19] Felicetti M, Cazzador D, Padoan R, Pendolino AL, Faccioli C,
Nardello E, et al. Ear, nose and throat involvement in granulomato-
sis with polyangiitis: how it presents and how it determined disease
severity and long-term outcomes. Clin Rheumatol 2018;37:1075–83.
[20] Ono N, Niiro H, Ueda A, Sawabe T, Nishizaka H, Furugo I, et al. Char-
acteristics of MPO-ANCA-positive granulomatosis with polyangiitis: a
retrospective multi-center study in Japan. Rhematol Int 2015;35:555–9.
[21] Rahmattulla C, de Lind van Wijngaarden RA, Barden AE, Hauer HA,
Floßmann O, Jayne DR, et al. Renal function and ear, nose, throat in-
volvement in anti-neutrophil cytoplasmic antibody-associated vasculitis:
prospective data from the European Vasculitis Society clinical trials.
Rheumatol 2015;54:899–907.
889
[22] Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW,
Dadoniené J, et al. A randomized trial of maintenance therapy for vas-
culitis associated with antineutrophil cytoplasmic autoantibodies. N Engl
J Med 2003;349:36–44.
[23] Ozaki S, Atsumi T, Hayashi T, Ishizu A, Kobayashi S, Kuma-
gai S, et al. Severity-based treatment for Japanese patients with
MPO-ANCA-associated vascolitis: the JMAAV study. Mod Rheumatol
2012;22:394–404.
[24] Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P,
et al. Rituximab versus azathioprine for maintenance in ANCA-associ-
ated vasculitis. N Engl J Med 2014;371:1771–80.
[25] Wilde B, van Paassen P, Witzke O, Tervaert JWC. New pathophysio-
logical insights and treatment of ANCA-associated vasculitis. Kidney
Int 2011;79:599–612.
[26] Bischof A, Jaeger VK, Hadden RDM, Luqmani RA, Pröbstel AK,
Merkel PA, et al. Peripheral neuropathy in antineutrophil cytoplasmic
antibody-associated vasculitis: in sight from the DCVAS study. Neurol
Neuroimmunol Neuroimflamm 2019;6:e615.
[27] Sugiyama K, Sada K, Kurosawa M, Wada J, Makino H. Current status
of the treatment of microscopic polyangiitis and granulomatosis with
polyangiitis in Japan. Clin Exp Nephrol 2013;17:51–8.