| 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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Correct Answer
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