Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine...Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.展开更多
Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drug...Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drugs are associated with a variety of well-known side effects such as anti-neutrophil cytoplasmic antibody （ANCA）-positive vasculitis, granulocytopenia and aplastic anemia. Recently, an atypical hematological finding -- bone marrow plasmacytosis, related to the use of methimazole -- was reported twice in English literatures, but bone marrow plasmacytosis with the use of propylthiouracil （PTU） has hardly been reported so far. Herein we present a case of a patient with Graves＇ disease who was initially investigated for plasma cell dyscrasia but finally diagnosed as PTU-induced bone marrow plasmacytosis with granulocytopenia and ANCA-associated vasculitis.展开更多
Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomeruloneph...Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity展开更多
目的嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)中以血管炎为主要特征和以嗜酸性粒细胞浸润为主要特征的两种亚型分类标准尚未明确,本研究探讨EGPA两种亚型的准确、简明分类方式。方法回顾性收集2000...目的嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)中以血管炎为主要特征和以嗜酸性粒细胞浸润为主要特征的两种亚型分类标准尚未明确,本研究探讨EGPA两种亚型的准确、简明分类方式。方法回顾性收集2000年1月—2023年11月北京协和医院呼吸内科、全科医学科、风湿免疫科等多科确诊的EGPA患者临床资料,将患者根据单纯抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibodies,ANCA)、ANCA与组织病理活检结果相结合以及MIRRA标准三种分类方法分为不同的阳性组和阴性组,分析患者的临床特征。结果共纳入245例患者。根据ANCA结果分类时,阳性组51例,阴性组194例,相较于阴性组,阳性组患者的年龄(60.9比55.4)、NEUT%(59.12比51.87)、PLT(312.94比264.64)、ESR(55.40比27.62)、CRP(46.51比23.57)均较高,上述指标均达显著统计学意义;阳性组肾脏系统(47.1%比25.8%)和神经系统(72.5%比58.2%)的受累比例也显著高于阴性组。根据ANCA结果和组织病理活检结果分类时,阳性组88例,阴性组157例,两组间的分析结果与第一种分类方法一致。根据MIRRA标准分类时,MIRRA标准组患者161例,MIRRA非标准组患者84例,MIRRA标准组的NEUT%(55.39比49.58)显著高于MIRRA非标准组,而LY%(18.57比21.30)和HGB(128.80比136.36)显著低于MIRRA非标准组。结论根据ANCA结果可将EGPA患者分为血管炎亚型和嗜酸性粒细胞浸润亚型;尽管ANCA联合组织病理活检结果也可明确区分血管炎亚型与嗜酸性粒细胞浸润亚型,但由于病理活检存在风险,需根据患者的具体情况进行个体化评估;而MIRRA标准不能准确区分两类亚型。展开更多
文摘Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.
文摘Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drugs are associated with a variety of well-known side effects such as anti-neutrophil cytoplasmic antibody （ANCA）-positive vasculitis, granulocytopenia and aplastic anemia. Recently, an atypical hematological finding -- bone marrow plasmacytosis, related to the use of methimazole -- was reported twice in English literatures, but bone marrow plasmacytosis with the use of propylthiouracil （PTU） has hardly been reported so far. Herein we present a case of a patient with Graves＇ disease who was initially investigated for plasma cell dyscrasia but finally diagnosed as PTU-induced bone marrow plasmacytosis with granulocytopenia and ANCA-associated vasculitis.
文摘Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity
