Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis.
OBJECTIVE
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.
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Juyoung
J
Yoo
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Ho Jae
HJ
Kim
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Sung Soo
SS
Ahn
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Seung Min
SM
Jung
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Jason Jungsik
JJ
Song
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Yong-Beom
YB
Park
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea. sangwonlee@yuhs.ac.
Sang-Won
SW
Lee
D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000097:blood* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000097:blood* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000097:blood* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology*; D009195:Peroxidase / Q000276:immunology*; D010865:Pilot Projects; D011237:Predictive Value of Tests; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D066106:Seoul; D013997:Time Factors; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D009195:Peroxidase; D053146:Myeloblastin