Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patie...Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patient with American tegumentary leishmaniasis in Mendoza,Argentina,a non-endemic region.Patient concerns:A 43-year-old male was admitted to a tertiary care hospital in Mendoza,Argentina Republic with a history of progressive nasal discharge,septal perforation,facial pain,and pruritus.Despite treatment for presumed nasal myiasis and vasculitis with granulomatosis,symptoms persisted.Diagnosis:American tegumentary leishmaniasis.Interventions:Intravenous liposomal amphotericin B.Outcomes:Follow-up at 30 days showed no recurrence of symptoms with a remarkable clinical improvement of the nasal lesion.Lessons:This case sheds light on the necessity of accurate identification for timely intervention and the need to recognize the diverse manifestations of American tegumentary leishmaniasis to avoid misdiagnosis.展开更多
Rituximab is a monoclonal antibody that targets CD20, which is a specific B-cell surface antigen. It was the first monoclonal antibody that was approved for the treatment of non-Hodgkin lymphoma, rheumatoid arthritis,...Rituximab is a monoclonal antibody that targets CD20, which is a specific B-cell surface antigen. It was the first monoclonal antibody that was approved for the treatment of non-Hodgkin lymphoma, rheumatoid arthritis, and other cutaneous lymphoid malignancies. There are many off-label uses of rituximab, such as systemic lupus erythematosus, autoimmune hemolytic anemia, multiple sclerosis, graft-versus-host disease, chronic lymphocytic leukemia, and chronic immune-mediated thrombocytopenia. Among the rare side effects associated with rituximab treatment is vasculitis, more specifically, leukocytoclastic vasculitis. Here, we describe a 21-year-old Saudi female with leukocytoclastic vasculitis occurring three months after treatment with rituximab.展开更多
Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multi...Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multiple organ systems throughout the body.Our knowledge of the pathogenesis of AAV has increased considerably in recent years,involving cellular,molecular and genetic factors.Because of the controlled environment with no other confounding factors,animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects.However,the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients.The aim of this review is to overview the current status of animal models for AAV,outline the pros and cons of methods,and propose potential directions for future research.展开更多
BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and rela...BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.展开更多
Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease cl...Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease classification based on ANCA subtype helps to differentiate clinical phenotypes. We investigated the clinical features and outcomes of patients based on ANCA serotype classification. Methods: Clinical and laboratory data were collected retrospectively. We compared clinical manifestations and organ involvement based on serotype. The risk factors for death and renal survival were investigated with univariate and multivariate Cox regression models. Results: Patients with MPO-ANCA were predominant, whose median age and lung involvement at diagnosis was higher than that of the PR3-ANCA patients. Compared to the AAV patients without renal involvement, those with renal involvement have older, anemia, low complement C3, and hypoproteinemia, more likely to have cardiovascular and abdominal involvement, and have less lung involvement. Multivariate Cox analysis revealed that age ≥ 65 years (HR = 2.611, p p = 0.019), BVAS ≥ 15 (HR = 1.943, p = 0.001), low C3 (HR = 1.696, p = 0.008), and hypoproteinemia (HR = 1.438, p = 0.044) were associated with mortality. SCR ≥ 500 μmol/L (HR = 13.583, p p = 0.020), low C3 (HR = 1.506, p = 0.049) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR = 0.523, p = 0.003). Conclusions: Clinical manifestations varied by AAV categories. Age, SCR, BVAS, low C3 and hypoproteinemia at diagnosis were independent predictors of mortality. BVAS, low C3, SCR at diagnosis and immunosuppressive treatment were independently related to renal survival in ANCA positive patients.展开更多
The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressi...The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.展开更多
嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiits,EGPA)主要特征为嗜酸性粒细胞增多、肉芽肿形成、中小血管坏死性血管炎。EGPA患者因嗜酸性粒细胞浸润、嗜酸性粒细胞诱导的纤维化和高凝状态导致器官损伤,同时伴...嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiits,EGPA)主要特征为嗜酸性粒细胞增多、肉芽肿形成、中小血管坏死性血管炎。EGPA患者因嗜酸性粒细胞浸润、嗜酸性粒细胞诱导的纤维化和高凝状态导致器官损伤,同时伴过敏表现。本文围绕EGPA的发病机制、临床表现、分类标准变迁及治疗进展进行阐述,并对其诊断及治疗面临的临床困境提出建议与思考。展开更多
目的探讨高凝状态与IgA血管炎肾炎(immunoglobulin A vasculitis with nephritis,IgAVN)患儿尿蛋白和肾脏病理损伤之间的关系。方法根据凝血功能结果,将349例IgAV患儿分为高凝组(52例)和非高凝组(297例),比较两组间尿蛋白和肾脏病理特征...目的探讨高凝状态与IgA血管炎肾炎(immunoglobulin A vasculitis with nephritis,IgAVN)患儿尿蛋白和肾脏病理损伤之间的关系。方法根据凝血功能结果,将349例IgAV患儿分为高凝组(52例)和非高凝组(297例),比较两组间尿蛋白和肾脏病理特征,并分析IgAVN患儿高凝状态形成的影响因素。结果高凝组患儿尿红细胞计数、24 h尿蛋白定量、尿蛋白/肌酐、尿免疫球蛋白G/肌酐、尿N-乙酰β-D-氨基葡萄糖苷酶水平高于非高凝组(P<0.05);高凝组患儿肾脏病理分级为Ⅲ~Ⅳ级、弥漫性系膜增生、毛细血管内皮细胞增生、肾小球新月体形成>25%比例高于非高凝组(P<0.05)。多因素logistic回归分析结果显示,毛细血管内皮细胞增生、肾小球新月体形成>25%是IgAVN患儿高凝状态形成的影响因素(P<0.05)。结论伴高凝状态IgAVN患儿的肾损伤更为严重,肾小球新月体形成>25%和毛细血管内皮细胞增生是进一步加重IgAVN高凝状态的重要因素。展开更多
基金supported by the Consejo Nacional de Investigaciones Cientıfícas y Técnicas(CONICET)(PIP 2021-2023/11220200102779CO).
文摘Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patient with American tegumentary leishmaniasis in Mendoza,Argentina,a non-endemic region.Patient concerns:A 43-year-old male was admitted to a tertiary care hospital in Mendoza,Argentina Republic with a history of progressive nasal discharge,septal perforation,facial pain,and pruritus.Despite treatment for presumed nasal myiasis and vasculitis with granulomatosis,symptoms persisted.Diagnosis:American tegumentary leishmaniasis.Interventions:Intravenous liposomal amphotericin B.Outcomes:Follow-up at 30 days showed no recurrence of symptoms with a remarkable clinical improvement of the nasal lesion.Lessons:This case sheds light on the necessity of accurate identification for timely intervention and the need to recognize the diverse manifestations of American tegumentary leishmaniasis to avoid misdiagnosis.
文摘Rituximab is a monoclonal antibody that targets CD20, which is a specific B-cell surface antigen. It was the first monoclonal antibody that was approved for the treatment of non-Hodgkin lymphoma, rheumatoid arthritis, and other cutaneous lymphoid malignancies. There are many off-label uses of rituximab, such as systemic lupus erythematosus, autoimmune hemolytic anemia, multiple sclerosis, graft-versus-host disease, chronic lymphocytic leukemia, and chronic immune-mediated thrombocytopenia. Among the rare side effects associated with rituximab treatment is vasculitis, more specifically, leukocytoclastic vasculitis. Here, we describe a 21-year-old Saudi female with leukocytoclastic vasculitis occurring three months after treatment with rituximab.
基金the Ethics Committee of Tongji Hospital,Huazhong University of Science and Technology,China(TJ-IRB20191012).
文摘Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multiple organ systems throughout the body.Our knowledge of the pathogenesis of AAV has increased considerably in recent years,involving cellular,molecular and genetic factors.Because of the controlled environment with no other confounding factors,animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects.However,the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients.The aim of this review is to overview the current status of animal models for AAV,outline the pros and cons of methods,and propose potential directions for future research.
文摘BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.
文摘Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease classification based on ANCA subtype helps to differentiate clinical phenotypes. We investigated the clinical features and outcomes of patients based on ANCA serotype classification. Methods: Clinical and laboratory data were collected retrospectively. We compared clinical manifestations and organ involvement based on serotype. The risk factors for death and renal survival were investigated with univariate and multivariate Cox regression models. Results: Patients with MPO-ANCA were predominant, whose median age and lung involvement at diagnosis was higher than that of the PR3-ANCA patients. Compared to the AAV patients without renal involvement, those with renal involvement have older, anemia, low complement C3, and hypoproteinemia, more likely to have cardiovascular and abdominal involvement, and have less lung involvement. Multivariate Cox analysis revealed that age ≥ 65 years (HR = 2.611, p p = 0.019), BVAS ≥ 15 (HR = 1.943, p = 0.001), low C3 (HR = 1.696, p = 0.008), and hypoproteinemia (HR = 1.438, p = 0.044) were associated with mortality. SCR ≥ 500 μmol/L (HR = 13.583, p p = 0.020), low C3 (HR = 1.506, p = 0.049) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR = 0.523, p = 0.003). Conclusions: Clinical manifestations varied by AAV categories. Age, SCR, BVAS, low C3 and hypoproteinemia at diagnosis were independent predictors of mortality. BVAS, low C3, SCR at diagnosis and immunosuppressive treatment were independently related to renal survival in ANCA positive patients.
文摘The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.
文摘嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiits,EGPA)主要特征为嗜酸性粒细胞增多、肉芽肿形成、中小血管坏死性血管炎。EGPA患者因嗜酸性粒细胞浸润、嗜酸性粒细胞诱导的纤维化和高凝状态导致器官损伤,同时伴过敏表现。本文围绕EGPA的发病机制、临床表现、分类标准变迁及治疗进展进行阐述,并对其诊断及治疗面临的临床困境提出建议与思考。
文摘目的探讨高凝状态与IgA血管炎肾炎(immunoglobulin A vasculitis with nephritis,IgAVN)患儿尿蛋白和肾脏病理损伤之间的关系。方法根据凝血功能结果,将349例IgAV患儿分为高凝组(52例)和非高凝组(297例),比较两组间尿蛋白和肾脏病理特征,并分析IgAVN患儿高凝状态形成的影响因素。结果高凝组患儿尿红细胞计数、24 h尿蛋白定量、尿蛋白/肌酐、尿免疫球蛋白G/肌酐、尿N-乙酰β-D-氨基葡萄糖苷酶水平高于非高凝组(P<0.05);高凝组患儿肾脏病理分级为Ⅲ~Ⅳ级、弥漫性系膜增生、毛细血管内皮细胞增生、肾小球新月体形成>25%比例高于非高凝组(P<0.05)。多因素logistic回归分析结果显示,毛细血管内皮细胞增生、肾小球新月体形成>25%是IgAVN患儿高凝状态形成的影响因素(P<0.05)。结论伴高凝状态IgAVN患儿的肾损伤更为严重,肾小球新月体形成>25%和毛细血管内皮细胞增生是进一步加重IgAVN高凝状态的重要因素。
