ANCA-negative limited Wegener's granulomatosis

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.