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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Arthritis+Res+Ther
2014 ; 16
(2
): R101
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Classification and characteristics of Japanese patients with antineutrophil
cytoplasmic antibody-associated vasculitis in a nationwide, prospective,
inception cohort study
#MMPMID24758294
Sada KE
; Yamamura M
; Harigai M
; Fujii T
; Dobashi H
; Takasaki Y
; Ito S
; Yamada H
; Wada T
; Hirahashi J
; Arimura Y
; Makino H
Arthritis Res Ther
2014[Apr]; 16
(2
): R101
PMID24758294
show ga
INTRODUCTION: We investigated the clinical and serological features of patients
with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in
Japan using data from a nationwide, prospective, inception cohort study. METHODS:
In total, 156 Japanese patients with newly diagnosed AAV were classified
according to the European Medicines Agency (EMEA) algorithm with exploratory
surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined
as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical
and serological features were evaluated. RESULTS: Using the EMEA algorithm, we
identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis
(EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with
microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%)
with unclassifiable vasculitis. The average ages of patients with EGPA
(male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22)
were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA
and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for
GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable,
respectively. According to the Birmingham Vasculitis Activity Score (BVAS),
cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in
EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations
(66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and
GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51
mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The
percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for
MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n?=?61) had
significantly lower BVAS (P?=?0.019) with fewer ear, nose, and throat and
cardiovascular manifestations than patients without ILD (n?=?95). CONCLUSIONS:
MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients,
and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important
clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis
with MPO-ANCA positivity and ILD may represent a novel variant of MPA. TRIAL
REGISTRATION: The University Hospital Medical Information Network Clinical Trials
Registry: UMIN000001648. Registered 28 February 2009.