A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener...A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.展开更多
Wegener’s Granulomatosis (WG)is a multi-system disorder characterized by necrotizing granulomas in the upper and/or lower respiratory tract, with or without focal
Abstract:Objective To map the epitopes on Wegener's granulomatosis autoantigen proteinase 3.Methods Antigenicity of proteinase 3 was studied with Western blot analysis in which proteinase 3 was prepared under redu...Abstract:Objective To map the epitopes on Wegener's granulomatosis autoantigen proteinase 3.Methods Antigenicity of proteinase 3 was studied with Western blot analysis in which proteinase 3 was prepared under reducing and non-reducing conditions. Two anti-proteinase 3 monoclonal antibodies, HZ1F12 and HZ1H3, were used to inhibit each other and to inhibit 22 anti-proteinase 3 positive sera from patients with Wegener's granulomatosis in competitive inhibition enzyme-linked immunosorbent assays (ELISA) and Western blot analysis. Results All monoclonal antibodies and patient sera recognized proteinase 3 under non-reducing conditions in Western blot analysis. HZ1F12 was inhibited 74% by HZ1H3. 10/22 (46%) sera were completely or partially inhibited by HZ1F12; 9/22 (41%) sera were partially inhibited by HZ1H3; and 6/22 (27%) were inhibited by both monoclonal antibodies. In inhibition Western blot analysis, the binding of patient sera to proteinase 3 could be inhibited by HZ1F12. Conclusions The epitopes of Wegener's granulomatosis autoantigen were conformational. Anti-proteinase 3 monoclonal HZ1F12 and HZ1H3 recognized similar or overlapping epitopes on the proteinase 3 molecule. Epitopes of proteinase 3 recognized by anti-proteinase 3 positive sera were not restricted.展开更多
文摘A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.
文摘Wegener’s Granulomatosis (WG)is a multi-system disorder characterized by necrotizing granulomas in the upper and/or lower respiratory tract, with or without focal
文摘Abstract:Objective To map the epitopes on Wegener's granulomatosis autoantigen proteinase 3.Methods Antigenicity of proteinase 3 was studied with Western blot analysis in which proteinase 3 was prepared under reducing and non-reducing conditions. Two anti-proteinase 3 monoclonal antibodies, HZ1F12 and HZ1H3, were used to inhibit each other and to inhibit 22 anti-proteinase 3 positive sera from patients with Wegener's granulomatosis in competitive inhibition enzyme-linked immunosorbent assays (ELISA) and Western blot analysis. Results All monoclonal antibodies and patient sera recognized proteinase 3 under non-reducing conditions in Western blot analysis. HZ1F12 was inhibited 74% by HZ1H3. 10/22 (46%) sera were completely or partially inhibited by HZ1F12; 9/22 (41%) sera were partially inhibited by HZ1H3; and 6/22 (27%) were inhibited by both monoclonal antibodies. In inhibition Western blot analysis, the binding of patient sera to proteinase 3 could be inhibited by HZ1F12. Conclusions The epitopes of Wegener's granulomatosis autoantigen were conformational. Anti-proteinase 3 monoclonal HZ1F12 and HZ1H3 recognized similar or overlapping epitopes on the proteinase 3 molecule. Epitopes of proteinase 3 recognized by anti-proteinase 3 positive sera were not restricted.