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Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis

 J. Yoo  ;  H. Kim  ;  S. Ahn  ;  S. Jung  ;  J. Song  ;  Y. Park  ;  S. Lee 
 Clinical and Experimental Rheumatology, Vol.35(Suppl. 103) : 111-118, 2017 
Journal Title
 Clinical and Experimental Rheumatology 
Issue Date
Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic ; Biomarkers/blood ; Churg-Strauss Syndrome/blood* ; Churg-Strauss Syndrome/diagnosis ; Churg-Strauss Syndrome/immunology ; Churg-Strauss Syndrome/therapy ; Disease-Free Survival ; Female ; Granulomatosis with Polyangiitis/blood* ; Granulomatosis with Polyangiitis/diagnosis ; Granulomatosis with Polyangiitis/immunology ; Granulomatosis with Polyangiitis/therapy ; Humans ; Male ; Microscopic Polyangiitis/blood* ; Microscopic Polyangiitis/diagnosis ; Microscopic Polyangiitis/immunology ; Microscopic Polyangiitis/therapy ; Middle Aged ; Myeloblastin/immunology* ; Peroxidase/immunology* ; Pilot Projects ; Predictive Value of Tests ; Recurrence ; Remission Induction ; Retrospective Studies ; Risk Factors ; Seoul ; Time Factors ; Treatment Outcome
OBJECTIVES: We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis, and investigated clinical and prognostic features. METHODS: We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission. We compared clinical manifestations, initial Birmingham vasculitis activity score (BVAS) and five factor score (FFS), and relapse rates. RESULTS: Patients with ANCA-negative vasculitis had the youngest mean age at diagnosis (50.0 years old) among AAV categories. General, cutaneous and renal manifestations were commonly observed in MPO-ANCA vasculitis, while mucous membrane, eye, ear nose throat (ENT) and renal manifestations were often documented in PR3-ANCA vasculitis. ENT manifestation was also frequently observed in ANCA-negative vasculitis. However, there were no significant differences in pulmonary and nervous system manifestations among 3 AAV categories. There were no significant differences in cumulative relapse free survival according to the presence of MPO-ANCA or PR3-ANCA or no ANCA. Meanwhile, initial BVAS or BVAS for GPA ≥13.5 in MPO-ANCA vasculitis and initial FFS (1996) ≥1 in MPO-ANCA and ANCA-negative vasculitis were significant predictors of relapse of each AAV category. CONCLUSIONS: Clinical manifestations varied AAV categories, and neither MPO-ANCA nor PR3-ANCA significantly affected relapse of AAV. Initial BVAS or BVAS for GPA and FFS (1996) helped to predict relapse of specified AAV categories.
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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
안성수(Ahn, Sung Soo) ORCID logo https://orcid.org/0000-0002-9002-9880
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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