Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally co...Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally compatible with the causative agent (whether there is a deep venous catheter or not). On the other hand and according to the 2012 Revised Chapel Hill Classification, granulomatosis with polyangiitis GPA is classified as a vasculitis associated with antineutrophil cytoplasmic antibodies ANCA. It is a systemic disease characterized by the anatomopathological aspect of granuloma. We report the case of a patient who presented an atypical and a very rare revealing mode of GPA which was a bronchopulmonary candidiasis complicated by candidemia. Despite its controversy, the combination in the acute phase of antifungal treatment based on intravenous voriconazole and glucocorticoid therapy has made it possible to control candidemia and calm vasculitis.展开更多
Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis...Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.展开更多
目的研究并比较肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)和显微镜下多血管炎(microscopic polyangiitis,MPA)死亡病例的死因及临床特点。方法回顾性分析北京协和医院因MPA或GPA住院的病例,评估疾病活动度,并统计分析两...目的研究并比较肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)和显微镜下多血管炎(microscopic polyangiitis,MPA)死亡病例的死因及临床特点。方法回顾性分析北京协和医院因MPA或GPA住院的病例,评估疾病活动度,并统计分析两组死因及疾病特点。结果 455例诊断为GPA或MPA的住院患者中,死亡49例(GPA 15例,MPA 34例),MPA病死率明显高于GPA(14.6%vs.6.8%,P<0.05)。GPA死亡患者的平均年龄明显低于MPA[(47.2±17.7)岁vs.(69.4±9.3)岁,P<0.001]。MPA生存时间中位数明显低于GPA(3.85个月vs.10.00个月,P<0.05)。起病3个月内死亡的MPA患者明显高于GPA(44.1%vs.6.7%,P<0.05)。MPA患者五因子评分明显高于GPA[(3.2±1.0)vs.(1.5±0.5),P<0.001]。GPA患者首要死因是原发病活动导致出血(53.4%),而MPA首要死因是感染(55.9%)。两组死因累及的首要系统均为呼吸系统,MPA占82.4%,GPA占66.7%。MPA组合并肺间质病变明显高于GPA(44.1%vs.13.3%,P<0.05);而合并耳鼻喉表现明显低于GPA组(2.9%vs.66.7%,P<0.001)。肺部感染病原分析显示,最常见为细菌感染,其次是真菌感染;且MPA组合并真菌感染(58.8%vs.26.7%,P<0.05)及混合感染(44.1%vs.13.3%,P<0.05)的发生率均高于GPA组。结论与GPA相比,MPA病死率高,且MPA患者病情重,进展快,易发生严重感染。展开更多
Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. ...Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCAassociated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.展开更多
Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but ...Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.展开更多
肉芽肿性多血管炎（granulomatosis with poly—angiitis，GPA）是以全身多器官、多系统的小血管受累为主的自身免疫性疾病，病理表现以坏死性肉芽肿血管炎为特征，病变累及小动脉、静脉及毛细血管为主，属于抗中性粒细胞胞浆抗体（ANC...肉芽肿性多血管炎（granulomatosis with poly—angiitis，GPA）是以全身多器官、多系统的小血管受累为主的自身免疫性疾病，病理表现以坏死性肉芽肿血管炎为特征，病变累及小动脉、静脉及毛细血管为主，属于抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（ANCA—associated vasculitis，AAV）。展开更多
目的分析以鼻部症状为首发表现的肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的临床诊断与治疗特点。方法收集2005—2019年就诊于山东第一医科大学附属省立医院耳鼻咽喉头颈外科以鼻部症状为首发表现并诊断为GPA的18例患...目的分析以鼻部症状为首发表现的肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的临床诊断与治疗特点。方法收集2005—2019年就诊于山东第一医科大学附属省立医院耳鼻咽喉头颈外科以鼻部症状为首发表现并诊断为GPA的18例患者的临床资料,其中男性8例,女性10例,年龄5~68岁。所有患者均完善鼻内镜检查、影像学检查、实验室检查、免疫学检查及鼻腔黏膜组织病理学检查。采取糖皮质激素联合环磷酰胺治疗,随访2~15年。采用描述性统计学方法进行分析。结果18例患者均存在鼻部首发症状,表现为鼻塞、流涕、鼻出血等。鼻内镜检查可见鼻腔黏膜肿胀、糜烂、干痂、出血,有6例发生鼻中隔穿孔。鼻窦CT表现为鼻窦内高密度影或上颌窦骨质明显增生、硬化。肺部CT结果呈结节灶或斑片状浸润者12例,空洞6例。实验室检查结果中抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibodies,ANCA)阳性13例,ANCA阴性5例。随访过程中,13例患者症状得以控制并存活至今,2例死于病情发展,1例拒绝治疗后死亡,2例失访。结论GPA有以鼻部症状为首发的临床表现,早期诊断、采用糖皮质激素联合环磷酰胺的方法早期治疗可以有效提高生存率。展开更多
文摘Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally compatible with the causative agent (whether there is a deep venous catheter or not). On the other hand and according to the 2012 Revised Chapel Hill Classification, granulomatosis with polyangiitis GPA is classified as a vasculitis associated with antineutrophil cytoplasmic antibodies ANCA. It is a systemic disease characterized by the anatomopathological aspect of granuloma. We report the case of a patient who presented an atypical and a very rare revealing mode of GPA which was a bronchopulmonary candidiasis complicated by candidemia. Despite its controversy, the combination in the acute phase of antifungal treatment based on intravenous voriconazole and glucocorticoid therapy has made it possible to control candidemia and calm vasculitis.
文摘Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.
文摘Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCAassociated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.
文摘Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.
文摘肉芽肿性多血管炎（granulomatosis with poly—angiitis，GPA）是以全身多器官、多系统的小血管受累为主的自身免疫性疾病，病理表现以坏死性肉芽肿血管炎为特征，病变累及小动脉、静脉及毛细血管为主，属于抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（ANCA—associated vasculitis，AAV）。
文摘目的分析以鼻部症状为首发表现的肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)的临床诊断与治疗特点。方法收集2005—2019年就诊于山东第一医科大学附属省立医院耳鼻咽喉头颈外科以鼻部症状为首发表现并诊断为GPA的18例患者的临床资料,其中男性8例,女性10例,年龄5~68岁。所有患者均完善鼻内镜检查、影像学检查、实验室检查、免疫学检查及鼻腔黏膜组织病理学检查。采取糖皮质激素联合环磷酰胺治疗,随访2~15年。采用描述性统计学方法进行分析。结果18例患者均存在鼻部首发症状,表现为鼻塞、流涕、鼻出血等。鼻内镜检查可见鼻腔黏膜肿胀、糜烂、干痂、出血,有6例发生鼻中隔穿孔。鼻窦CT表现为鼻窦内高密度影或上颌窦骨质明显增生、硬化。肺部CT结果呈结节灶或斑片状浸润者12例,空洞6例。实验室检查结果中抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibodies,ANCA)阳性13例,ANCA阴性5例。随访过程中,13例患者症状得以控制并存活至今,2例死于病情发展,1例拒绝治疗后死亡,2例失访。结论GPA有以鼻部症状为首发的临床表现,早期诊断、采用糖皮质激素联合环磷酰胺的方法早期治疗可以有效提高生存率。
