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Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis

 Juyoung Yoo  ;  Ho Jae Kim  ;  Seung Min Jung  ;  Jason Jungsik Song  ;  Yong‐Beom Park  ;  Sang‐Won Lee 
 International Journal of Rheumatic Diseases, Vol.20(10) : 1593-1605, 2017 
Journal Title
 International Journal of Rheumatic Diseases 
Issue Date
Aged ; Antibodies, Antineutrophil Cytoplasmic/blood* ; Biomarkers/blood ; Biopsy ; Chi-Square Distribution ; Decision Support Techniques* ; Disease-Free Survival ; Drug Resistance ; Enzyme-Linked Immunosorbent Assay ; Female ; Granulomatosis with Polyangiitis/blood ; Granulomatosis with Polyangiitis/diagnosis* ; Granulomatosis with Polyangiitis/drug therapy* ; Granulomatosis with Polyangiitis/immunology ; Humans ; Immunosuppressive ; Agents/therapeutic use* ; Logistic Models ; Male ; Medical Records ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Predictive Value of Tests ; Proportional Hazards Models ; Recurrence ; Retrospective Studies ; Risk Factors ; Severity of Illness Index ; Time Factors ; Treatment Outcome
FFS ; BVAS for GPA ; granulomatosis with polyangiitis ; predictors ; refractory disease ; relapse
AIM: We investigated whether clinical manifestations, anti-neutrophil cytoplasmic antibodies (ANCAs), Birmingham vasculitis activity score (BVAS) for granulomatosis with polyangiitis (GPA) and five factor score (FFS) at diagnosis can predict relapse or refractory disease in 30 histology-proven GPA patients with follow-up duration ≥ 12 weeks. METHODS: We reviewed the medical records of 30 GPA patients. We collected clinical data, ANCAs, BVAS for GPA, FFSs at diagnosis, and we compared variables between the two groups based on relapse or refractory disease. The optimal cut-offs were extrapolated. Multivariate logistic regression and Cox hazard model analyses were conducted to identify predictors of refractory disease. RESULTS: The mean age and follow-up duration of patients were 63.3 years old and 45.2 months. The mean initial BVAS for GPA, FFS (1996) and FFS (2009) were 5.4, 0.6 and 1.0. There were no significant predictors of relapse. The mean BVAS for GPA, FFS (1996) and FFS (2009) of patients with refractory disease were higher than those without (P < 0.05 for all). Patients having BVAS for GPA ≥ 9.5, FFS (1996) ≥ 2 and FFS (2009) ≥ 2 exhibited significantly enhanced risk of refractory disease than those without (relative risk 23.0, 11.0, and 55.0, respectively), and low cumulative refractory disease-free survival rates. Multivariate Cox hazard model analysis proved BVAS for GPA ≥ 9.5 was an independent predictor of refractory disease during the follow-up duration (odds ratio 12.892). CONCLUSION: BVAS for GPA ≥ 9.5 was an independent predictor of refractory disease during follow-up duration ≥ 12 weeks.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
박용범(Park, Yong Beom)
송정식(Song, Jungsik Jason) ORCID logo https://orcid.org/0000-0003-0662-7704
유주영(Yoo, Juyoung) ORCID logo https://orcid.org/0000-0001-8882-1695
이상원(Lee, Sang Won) ORCID logo https://orcid.org/0000-0002-8038-3341
정승민(Jung, Seung Min ) ORCID logo https://orcid.org/0000-0003-3465-2181
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