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Mod Rheumatol. 2016 Sep;26(5):730-7. doi: 10.3109/14397595.2016.1140274. Epub 2016 Mar 11.

Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.

Author information

1
a Department of Nephrology, Rheumatology, Endocrinology and Metabolism , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan .
2
b Department of Pharmacovigilance .
3
c Department of Rheumatology, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University , Tokyo , Japan .
4
d Department of Rheumatology and Clinical Immunology , Saitama Medical Center, Saitama Medical University , Saitama , Japan .
5
e Division of Rheumatology, Endocrinology and Nephrology at the Graduate School of Medicine , Hokkaido University , Hokkaido , Japan .
6
f Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine , Miyazaki University , Miyazaki , Japan .
7
g Department of Nephrology , Fujita Health University School of Medicine , Aichi , Japan .
8
h Department of Internal Medicine and Rheumatology , Juntendo University School of Medicine, Tokyo , Japan .
9
i Division of Rheumatology and Nephrology, Department of Internal Medicine , Aichi Medical University School of Medicine , Aichi , Japan .
10
j Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology , Tokyo , Japan .
11
k Department of Nephrology , Tokyo Medical University Ibaraki Medical Center , Ibaraki , Japan .
12
l Department of Nephrology, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan .
13
m Department of Respiratory Medicine , Toho University Omori Medical Center , Tokyo , Japan .
14
n Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Kagawa , Japan .
15
o Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine , Aichi , Japan .
16
p Faculty of Health Sciences , Hokkaido University , Hokkaido , Japan .
17
q Department of Chronic Kidney Disease and Peritoneal Dialysis , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan .
18
r Department of Pathology , Keio University School of Medicine , Tokyo , Japan .
19
s Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine , Tokyo , Japan , and.
20
t Okayama University Hospital , Okayama , Japan.

Abstract

OBJECTIVE:

To compare disease severity classification systems for six-month outcome prediction in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

METHODS:

Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated.

RESULTS:

According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease. According to the rapidly progressive glomerulonephritis (RPGN) clinical grading system, the patients were divided as follows: 60, grade I; 178, grade II; 66, grade III; and 12, grade IV. According to the Five-Factor Score (FFS) 2009, 103, 109, and 109 patients had ≤1, 2, and ≥3 points, respectively. No significant difference in remission rates was found in any severity classification. The overall and ESRD-free survival rates significantly differed between grades I/II, III, and IV, regardless of renal involvement. Severe disease was a good predictor of six-month overall and ESRD-free survival. The FFS 2009 was useful to predict six-month ESRD-free survival but not overall survival.

CONCLUSIONS:

The RPGN grading system was more useful to predict six-month overall and ESRD-free survival than the EUVAS-defined severity or FFS 2009.

KEYWORDS:

Antineutrophil cytoplasmic antibody-associated vasculitis; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Inception cohort; Microscopic polyangiitis

PMID:
26873424
DOI:
10.3109/14397595.2016.1140274
[Indexed for MEDLINE]
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