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: 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.
