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종결 Case No. 314 2013-09-02~2013-09-30
출제자 : 차지훈 Hit : 351
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Period 2013-09-02~2013-09-30
Diagnosis Wegener's granulomatosis
Clinical information M/45 CC: chronic sinusitis, 진단은?
Discussion 답: Wegener's granulomatosis (WG) or ANCA-associated granulomatous vasculitis or granulomatosis with polyangiitis (GPA)
해설
Name changed: Wegener's granulomatosis (WG)  ANCA-associated granulomatous vasculitis (2006)  granulomatosis with polyangiitis (GPA) (ACR, 2011)

Autoimmune necrotizing granulomatous vasculitis involving Small and medium sized vessels
- involving upper & lower respiratory tract, kidney, skin, joint
- H&N involvement in 72-100% of patients
- 40-60 years & M > F
- positive cANCA (cytoplasmic anti-neutrophil cytoplasmic antibody, anti-proteinase 3 (PR3)), 85-98% specificity, 10-50% may negative

Imaging
- nodular soft tissue (may hypointense on T2WI compared to inflammed mucosa) in nose with mucosal ulceration
- nasal septal perforation (lead to saddle nose deformity), turbinate/lateral nasal wall destruction
- associated with chronic sinusitis, sclerotic to destructive sinus wall change, periantral soft tissue infiltration
- orbital invasion : most common extrasinonasal H&N site

Treatment
Steroid, cyclophosphamide
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