J Korean Med Sci.  2009 Apr;24(2):269-274. 10.3346/jkms.2009.24.2.269.

Clinical Features and Outcomes of Microscopic Polyangiitis in Korea

• Oh JS
• Lee CK
• Kim YG
• Nah SS
• Moon HB
• Yoo B

• KMID: 1779129
• DOI: http://doi.org/10.3346/jkms.2009.24.2.269

Abstract

Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.

Keyword

Microscopic Polyangiitis; Antibodies, Antineutrophil Cytoplasmic; Vasculitis

MeSH Terms

Adult
Age Factors
Aged
Aged, 80 and over
Antibodies, Antineutrophil Cytoplasmic/blood
Cyclophosphamide/therapeutic use
Drug Therapy, Combination
Female
Hemorrhage/etiology
Humans
Kidney Failure/etiology
Korea
Lung Diseases/etiology
Male
Middle Aged
Polyarteritis Nodosa/*diagnosis/drug therapy/mortality
Pulmonary Alveoli/blood supply/pathology
Retrospective Studies
Steroids/therapeutic use
Survival Analysis
Treatment Outcome

Figure

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  Fig. 1 Kaplan-Meier survival curve for the 18 patients with microscopic polyangiitis.

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  Fig. 2 Kaplan-Meier survival curves for the 18 patients with microscopic polyangiitis relative to (A) Five-Factor Score (p not significant), (B) Birmingham Vasculitis Activity Score (p=0.1), (C) age (p=0.16), and (D) presence or absence of alveolar hemorrhage (p=0.0016). Data were analyzed using the log-rank test.

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