The Korean Journal of Internal Medicine

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Original Article
Korean J Med. 2009;76(1):103-107.
항호중구세포질항체 음성을 보인 제한성 베게너육아종증 1예
송종규, 이은주, 하은실, 강은해, 이상엽, 인광호, 유세화
ANCA-negative limited Wegener’s granulomatosis
Jong Gyu Song, Eun Joo Lee, Eun Sil Ha, Eun Hae Kang, Sang Yeub Lee, Kwang Ho In, Se Hwa Yoo


Abstract
Wegener’s granulomatosis is a very rare autoimmune disease that forms inflammatory granulomas of the upper and lower respiratory tract, and causes necrotizing vasculitis by invading small vessels. Its etiology is uncertain, but antineutrophil cytoplasmic antibody (ANCA) is thought to play an important role in causing the inflammatory granuloma formation and vasculitis. The detection of ANCA is a valuable finding in diagnosing Wegener’s granulomatosis. However, in the limited type of Wegener’s granulomatosis, which lacks accompanying constitutional symptoms, the diagnostic value of ANCA is minimal, requiring careful interpretation of ANCA-negativity. We report a case diagnosed as limited-type Wegener’s granulomatosis through repeated biopsies despite ANCA negativity. (Korean J Med 76:S103-S107, 2009)

Keywords :ANCA, Wegener granulomatosis
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