Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicate...Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006.Results Of 19 patients,3 were men and 16 were women,with a mean age of 24.4±7.7 years old.All had positive antinuclear antibodies and low serum complement was found in 13 patients.All were anemic and 12 of them were thrombocytopenic.Impaired renal function was found in 17 patients with an average serum creatinine of 184.5±88.9 μmol/L.Severe intrarenal arteriolar lesion was found in all patients.Six patients had lupus vasculopathy,11 patients had renal thrombotic microangiopathy lesion,2 had severe arteriosclerosis.All patients received steroids and immunosuppressive drugs,15 received angiotensin-converting enzyme inhibitor(ACEI)/angiotensin receptor blocker(ARB)with resultant well-controlled blood pressure.Thrombocytopenia and hemolytic anemia resolved remarkably.The renal function improved or recovered in 14 of 17 patients,and 3 developed end-stage renal disease on maintenance dialysis.Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension.Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type.On the basis of immunosuppressive drugs and steroids to control systemic lupus activity,timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.展开更多
Introduction: Multiple myeloma is a haematological malignancy characterised by monoclonal plasma cell proliferation in the bone marrow, which synthesises a serum and/or urinary monoclonal protein, associated with one ...Introduction: Multiple myeloma is a haematological malignancy characterised by monoclonal plasma cell proliferation in the bone marrow, which synthesises a serum and/or urinary monoclonal protein, associated with one or more manifestations including (anaemia, hypercalcaemia, bone lesions, renal failure). The aim of our work was to determine the prevalence, clinico-biological, histological and therapeutic manifestations of renal damage during multiple myeloma. Methodology: This is a retrospective descriptive study spread over a period of 2 years from 01 January 2020 to 31 December 2021 carried out in the nephrology department of the louis pasteur hospital of the CH of Chartres. All patients treated for multiple myeloma with renal involvement were included, whether the renal involvement was revelatory or diagnosed during the follow-up of the multiple myeloma. Results: We collected 20 cases of myeloma. Renal involvement was present in 19 cases (95%). The mean age was 68.78 ± 10.77 years with extremes ranging from 44 to 82 years. Males were more prevalent, with an M/F sex ratio of 3.75. The most common antecedent condition was hypertension in 14 cases (73.7%) and diabetes in 4 cases (21.1%). The circumstances of discovery were renal failure in 16 cases (84.2%), followed by bone pain in 6 cases (31.6%). Renal involvement was dominated by renal failure in 18 cases (94.7%), hypercalcaemia in 14 cases (73.7%), para protein in 9 cases (47.4%), haematuria and leukocyturia in 5 cases (26.3%). On plasma protein electrophoresis, 11 cases (57.9%) had kappa light chain multiple myeloma, 8 cases (42.1%) had lambda light chain multiple myeloma. We noted 9 cases (69%) of myelomatous cylinder nephropathy at renal biopsy, Randall’s disease in 2 cases (15%), AL amyloidosis and acute tubular necrosis in 1 case (8%). First-line chemotherapy treatment was dominated by the combination of Velcade, cyclophosphamide and dexamethasone in 5 cases (79%). Conclusion: Renal involvement in myeloma is still common;it may be isolated and precede the first signs of myeloma by several months;the most common histological forms are myelomatous cylinder nephropathy and Randall’s disease.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
Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with ...Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. Methods： Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute ofNephrology, Sichuan Provincial People＇s Hospital between 2004 and 2012 were collected retrospectively. All patients were followed up for 2 years after diagnosis. Survivors were compared with nonsurvivors to identify the clinical baseline variables associated with mortality. Multivariate Cox regression model was used to determine the independent predictors of mortality. Results： Of the 123 patients, 46 （37.4%） died by the end of 2 years after diagnosis, with 41 （33.3%） patients dying within the first 12 months. In comparison with the survivors, Birmingham Vasculitis Activity Score （BVAS）, the incidence of pulmonary hemorrhage and digestive system （DS） involvement, serum creatinine, and erythrocyte sedimentation rate were significantly higher in nonsurvivors, whereas lymphocyte counts, hemoglobin, and complement 3 （C3） were significantly lower. Renal replacement therapy was more common in nonsurvivors. High BVAS （hazard ratio [HR] = 1.058, 95% confidence interval [CI]： 1.002-1. I17; P = 0.042）, pulmonary hemorrhage （MR = 1.970, 95% CI： 1.033-3.757; P = 0.04）, DS involvement （HR = 2.911,95% （7：1.212-6.911 ： P = 0.017）4 and serum creatinine 〉400 μmol/L （MR = 2.910, 95% CI： 1.271-6.664, P = 0.012） were independent predictors of death in patients with AAV-related renal injury. Conclusions： Patients with AAV-related renal injury have high early mortality. Those with high BVAS （particularly with pulmonary or DS involvement） and serious renal dysfunction should receive aggressive therapy and careful monitoring to reduce the occurrence of adverse events and improve prognosis.展开更多
文摘Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006.Results Of 19 patients,3 were men and 16 were women,with a mean age of 24.4±7.7 years old.All had positive antinuclear antibodies and low serum complement was found in 13 patients.All were anemic and 12 of them were thrombocytopenic.Impaired renal function was found in 17 patients with an average serum creatinine of 184.5±88.9 μmol/L.Severe intrarenal arteriolar lesion was found in all patients.Six patients had lupus vasculopathy,11 patients had renal thrombotic microangiopathy lesion,2 had severe arteriosclerosis.All patients received steroids and immunosuppressive drugs,15 received angiotensin-converting enzyme inhibitor(ACEI)/angiotensin receptor blocker(ARB)with resultant well-controlled blood pressure.Thrombocytopenia and hemolytic anemia resolved remarkably.The renal function improved or recovered in 14 of 17 patients,and 3 developed end-stage renal disease on maintenance dialysis.Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension.Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type.On the basis of immunosuppressive drugs and steroids to control systemic lupus activity,timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.
文摘Introduction: Multiple myeloma is a haematological malignancy characterised by monoclonal plasma cell proliferation in the bone marrow, which synthesises a serum and/or urinary monoclonal protein, associated with one or more manifestations including (anaemia, hypercalcaemia, bone lesions, renal failure). The aim of our work was to determine the prevalence, clinico-biological, histological and therapeutic manifestations of renal damage during multiple myeloma. Methodology: This is a retrospective descriptive study spread over a period of 2 years from 01 January 2020 to 31 December 2021 carried out in the nephrology department of the louis pasteur hospital of the CH of Chartres. All patients treated for multiple myeloma with renal involvement were included, whether the renal involvement was revelatory or diagnosed during the follow-up of the multiple myeloma. Results: We collected 20 cases of myeloma. Renal involvement was present in 19 cases (95%). The mean age was 68.78 ± 10.77 years with extremes ranging from 44 to 82 years. Males were more prevalent, with an M/F sex ratio of 3.75. The most common antecedent condition was hypertension in 14 cases (73.7%) and diabetes in 4 cases (21.1%). The circumstances of discovery were renal failure in 16 cases (84.2%), followed by bone pain in 6 cases (31.6%). Renal involvement was dominated by renal failure in 18 cases (94.7%), hypercalcaemia in 14 cases (73.7%), para protein in 9 cases (47.4%), haematuria and leukocyturia in 5 cases (26.3%). On plasma protein electrophoresis, 11 cases (57.9%) had kappa light chain multiple myeloma, 8 cases (42.1%) had lambda light chain multiple myeloma. We noted 9 cases (69%) of myelomatous cylinder nephropathy at renal biopsy, Randall’s disease in 2 cases (15%), AL amyloidosis and acute tubular necrosis in 1 case (8%). First-line chemotherapy treatment was dominated by the combination of Velcade, cyclophosphamide and dexamethasone in 5 cases (79%). Conclusion: Renal involvement in myeloma is still common;it may be isolated and precede the first signs of myeloma by several months;the most common histological forms are myelomatous cylinder nephropathy and Randall’s disease.
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Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. Methods： Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute ofNephrology, Sichuan Provincial People＇s Hospital between 2004 and 2012 were collected retrospectively. All patients were followed up for 2 years after diagnosis. Survivors were compared with nonsurvivors to identify the clinical baseline variables associated with mortality. Multivariate Cox regression model was used to determine the independent predictors of mortality. Results： Of the 123 patients, 46 （37.4%） died by the end of 2 years after diagnosis, with 41 （33.3%） patients dying within the first 12 months. In comparison with the survivors, Birmingham Vasculitis Activity Score （BVAS）, the incidence of pulmonary hemorrhage and digestive system （DS） involvement, serum creatinine, and erythrocyte sedimentation rate were significantly higher in nonsurvivors, whereas lymphocyte counts, hemoglobin, and complement 3 （C3） were significantly lower. Renal replacement therapy was more common in nonsurvivors. High BVAS （hazard ratio [HR] = 1.058, 95% confidence interval [CI]： 1.002-1. I17; P = 0.042）, pulmonary hemorrhage （MR = 1.970, 95% CI： 1.033-3.757; P = 0.04）, DS involvement （HR = 2.911,95% （7：1.212-6.911 ： P = 0.017）4 and serum creatinine 〉400 μmol/L （MR = 2.910, 95% CI： 1.271-6.664, P = 0.012） were independent predictors of death in patients with AAV-related renal injury. Conclusions： Patients with AAV-related renal injury have high early mortality. Those with high BVAS （particularly with pulmonary or DS involvement） and serious renal dysfunction should receive aggressive therapy and careful monitoring to reduce the occurrence of adverse events and improve prognosis.
