The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly pu...The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly published 2016 revision of the World Health Organization （WHO） classification of lymphoid neoplasms has officially accepted MBL as an independent disease entity Herein, we present a case of Wegener granulomatosis （WG） with MBL.展开更多
Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most co...Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms 展开更多
目的：提高对韦格纳肉芽肿（WG）并发高血糖症的认识。方法结合一例 WG 并发高血糖症患者的临床资料进行文献复习，对 WG 和高血糖症的内在联系进行分析。结果患者入院后在 CT 引导下行肺活检，病理检查结果提示：肉芽肿性炎，高度考虑...目的：提高对韦格纳肉芽肿（WG）并发高血糖症的认识。方法结合一例 WG 并发高血糖症患者的临床资料进行文献复习，对 WG 和高血糖症的内在联系进行分析。结果患者入院后在 CT 引导下行肺活检，病理检查结果提示：肉芽肿性炎，高度考虑为 WG。查胞浆型抗中性粒细胞胞浆抗体（cANCA）：阳性，核周型 ANCA（pANCA）：阴性，抗髓过氧化物酶抗体＜2．0，抗蛋白酶3抗体144．80。入院后监测血糖发现，餐前餐后血糖水平均明显高于正常，经激素＋环磷酰胺＋复方新诺明控制 WG 病情后血糖恢复正常。结论 WG 并发高血糖症原因可能为 WG 累及胰腺导致胰岛β细胞损伤、胰岛素内源性分泌减少。展开更多
目的观察鼻肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的组织病理形态及其IgG4免疫组织化学表达,探讨GPA与IgG4相关性疾病(IgG4 related disease,IgG4-RD)之间的关系。方法收集首都医科大学附属北京同仁医院病理科2012年1...目的观察鼻肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的组织病理形态及其IgG4免疫组织化学表达,探讨GPA与IgG4相关性疾病(IgG4 related disease,IgG4-RD)之间的关系。方法收集首都医科大学附属北京同仁医院病理科2012年1月~2016年12月间病理诊断的12例鼻GPA患者的临床资料,EnVision免疫组化二步法行CD38、CD138、IgG、IgG4染色。结果9例伴肺部异常改变;8例血清抗中性粒细胞胞浆抗体值升高;组织学评分,3分者8例,2分者1例,1分者3例;免疫组化结果显示8例IgG4+浆细胞计数超过10个/HPF,其中2例IgG4/IgG比值大于40%。结论 IgG4+浆细胞数量增多常见于鼻GPA,因GPA与IgG4-RD在组织形态学及临床特征上有重叠,对二者的鉴别非常重要,GPA的诊断需结合临床表现、影像学、血清学及病理学表现综合判定。展开更多
文摘The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly published 2016 revision of the World Health Organization （WHO） classification of lymphoid neoplasms has officially accepted MBL as an independent disease entity Herein, we present a case of Wegener granulomatosis （WG） with MBL.
文摘Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms
文摘目的：提高对韦格纳肉芽肿（WG）并发高血糖症的认识。方法结合一例 WG 并发高血糖症患者的临床资料进行文献复习，对 WG 和高血糖症的内在联系进行分析。结果患者入院后在 CT 引导下行肺活检，病理检查结果提示：肉芽肿性炎，高度考虑为 WG。查胞浆型抗中性粒细胞胞浆抗体（cANCA）：阳性，核周型 ANCA（pANCA）：阴性，抗髓过氧化物酶抗体＜2．0，抗蛋白酶3抗体144．80。入院后监测血糖发现，餐前餐后血糖水平均明显高于正常，经激素＋环磷酰胺＋复方新诺明控制 WG 病情后血糖恢复正常。结论 WG 并发高血糖症原因可能为 WG 累及胰腺导致胰岛β细胞损伤、胰岛素内源性分泌减少。
文摘目的观察鼻肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的组织病理形态及其IgG4免疫组织化学表达,探讨GPA与IgG4相关性疾病(IgG4 related disease,IgG4-RD)之间的关系。方法收集首都医科大学附属北京同仁医院病理科2012年1月~2016年12月间病理诊断的12例鼻GPA患者的临床资料,EnVision免疫组化二步法行CD38、CD138、IgG、IgG4染色。结果9例伴肺部异常改变;8例血清抗中性粒细胞胞浆抗体值升高;组织学评分,3分者8例,2分者1例,1分者3例;免疫组化结果显示8例IgG4+浆细胞计数超过10个/HPF,其中2例IgG4/IgG比值大于40%。结论 IgG4+浆细胞数量增多常见于鼻GPA,因GPA与IgG4-RD在组织形态学及临床特征上有重叠,对二者的鉴别非常重要,GPA的诊断需结合临床表现、影像学、血清学及病理学表现综合判定。
