Clinical features and outcomes of microscopic polyangiitis in Korea.
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.
Oh
Ji Seon
JS
Division of Allergy and Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Lee
Chang-Keun
CK
Kim
Yong Gil
YG
Nah
Seong-Su
SS
Moon
Hee-Bom
HB
Yoo
Bin
B
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007723:Korea; D008171:Lung Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D011650:Pulmonary Alveoli / Q000098:blood supply / Q000473:pathology; D051437:Renal Insufficiency / Q000209:etiology; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D016019:Survival Analysis; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D013256:Steroids; D003520:Cyclophosphamide