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Abstract：
BACKGROUND: Globally, there are regional and time-based variations in the prevalence, etiology, and prognosis of rapidly progressive glomerulonephritis (RPGN). Prognosis of RPGN is poor, with a higher risk of death and end stage renal disease (ESRD) even with immunosuppressive medications. In the Middle East and North Africa, the studies on this disease are very limited. Therefore, we determined the predictors of outcome of RPGN.
METHODS: We retrospectively assessed 101 adult patients over age of 18, diagnosed with RPGN based on renal biopsy illustrating crescents in ≥ 50% of the glomeruli. Patients who had crescents in their renal biopsies that were < 50% and those who refused to consent to a renal biopsy were excluded. We categorized the patients into 3 groups based on immunohistochemistry; type I, type II and type III. Then, depending on renal loss, we divided them into ESRD and non-ESRD groups. The clinical history and physical examination were retrieved. Additionally, 24-hour urine protein, urine analysis, renal function tests, serum albumin, complete blood count, antinuclear antibodies, anti-double stranded DNA antibodies, ANCA antibodies and serum complement levels were checked. Each patient underwent a kidney biopsy for immunohistochemistry and light microscopy. The percentage of crescentic glomeruli, number of sclerosed glomeruli, tertiary lymphoid organ (TLO), neutrophil infiltration, endocapillary or mesangial hypercellularity, interstitial fibrosis with tubular atrophy (IFTA) were analyzed. Primary outcomes (remission, ESRD and mortality) and secondary outcomes were assessed.
RESULTS: Type II was the most frequent cause of RPGN (47.5%), followed by type III (32.7%) and type I (19.8%). 32 patients (31.7%) died during follow up, whereas 60 patients (59.4%) developed ESRD. In 41 patients (40.6%), remission occurred. Oliguria, serum creatinine, and need for HD at presentation were significantly increased in ESRD group compared to non-ESRD group (P < 0.001 for each). Mesangial proliferation, IFTA, TLO formation, sclerotic glomeruli and fibrous crescents were also significantly increased in ESRD group in comparison to non-ESRD group (P < 0.001 for each). Glomerulosclerosis (P = 0.036), and IFTA (P = 0.008) were predictors of ESRD. Infections (P = 0.02), respiratory failure (P < 0.001), and heart failure (P = 0.004) were mortality risk factors.
CONCLUSIONS: Type II RPGN was the most common. Infection was the most frequent secondary outcome. Oliguria, glomerulosclerosis, the requirement for hemodialysis at presentation, IFTA and TLO formation were predictors of ESRD. Respiratory failure, heart failure and infections were significant predictors of mortality.
METHODS: We retrospectively assessed 101 adult patients over age of 18, diagnosed with RPGN based on renal biopsy illustrating crescents in ≥ 50% of the glomeruli. Patients who had crescents in their renal biopsies that were < 50% and those who refused to consent to a renal biopsy were excluded. We categorized the patients into 3 groups based on immunohistochemistry; type I, type II and type III. Then, depending on renal loss, we divided them into ESRD and non-ESRD groups. The clinical history and physical examination were retrieved. Additionally, 24-hour urine protein, urine analysis, renal function tests, serum albumin, complete blood count, antinuclear antibodies, anti-double stranded DNA antibodies, ANCA antibodies and serum complement levels were checked. Each patient underwent a kidney biopsy for immunohistochemistry and light microscopy. The percentage of crescentic glomeruli, number of sclerosed glomeruli, tertiary lymphoid organ (TLO), neutrophil infiltration, endocapillary or mesangial hypercellularity, interstitial fibrosis with tubular atrophy (IFTA) were analyzed. Primary outcomes (remission, ESRD and mortality) and secondary outcomes were assessed.
RESULTS: Type II was the most frequent cause of RPGN (47.5%), followed by type III (32.7%) and type I (19.8%). 32 patients (31.7%) died during follow up, whereas 60 patients (59.4%) developed ESRD. In 41 patients (40.6%), remission occurred. Oliguria, serum creatinine, and need for HD at presentation were significantly increased in ESRD group compared to non-ESRD group (P < 0.001 for each). Mesangial proliferation, IFTA, TLO formation, sclerotic glomeruli and fibrous crescents were also significantly increased in ESRD group in comparison to non-ESRD group (P < 0.001 for each). Glomerulosclerosis (P = 0.036), and IFTA (P = 0.008) were predictors of ESRD. Infections (P = 0.02), respiratory failure (P < 0.001), and heart failure (P = 0.004) were mortality risk factors.
CONCLUSIONS: Type II RPGN was the most common. Infection was the most frequent secondary outcome. Oliguria, glomerulosclerosis, the requirement for hemodialysis at presentation, IFTA and TLO formation were predictors of ESRD. Respiratory failure, heart failure and infections were significant predictors of mortality.
摘要：
背景：在全球范围内，患病率存在区域和基于时间的变化，病因学,快速进展性肾小球肾炎（RPGN）的预后。RPGN预后差,即使使用免疫抑制药物，死亡和终末期肾病（ESRD）的风险也更高。在中东和北非，对这种疾病的研究非常有限。因此,我们确定了RPGN结局的预测因子。
方法：我们回顾性评估了101例18岁以上的成人患者，这些患者根据肾活检诊断为RPGN，这些患者的肾小球中新月≥50%。排除在肾活检中具有<50％的新月的患者以及拒绝同意肾活检的患者。我们根据免疫组织化学将患者分为3组；I型，II型和III型。然后，取决于肾脏的损失，我们将他们分为ESRD和非ESRD组。检索临床病史和体格检查。此外,24小时尿蛋白，尿液分析,肾功能试验,血清白蛋白,全血细胞计数,抗核抗体,抗双链DNA抗体,检查ANCA抗体和血清补体水平。每位患者都接受了肾脏活检以进行免疫组织化学和光学显微镜检查。新月肾小球的百分比，硬化肾小球的数量，三级淋巴器官（TLO），中性粒细胞浸润，毛细血管内或系膜细胞增多，分析了间质纤维化伴肾小管萎缩(IFTA)。主要结果（缓解，评估ESRD和死亡率）和次要结局。
结果：II型是RPGN的最常见原因（47.5％），其次是III型（32.7％）和I型（19.8％）。32例（31.7%）在随访期间死亡，而60例患者（59.4%）发展为ESRD。41例患者（40.6%），出现缓解。少尿症,血清肌酐,与非ESRD组相比,ESRD组出现时对HD的需求显著增加(各P<0.001)。系膜增生,IFTA,TLO形成，与非ESRD组相比，ESRD组的硬化性肾小球和纤维新月也显着增加（每个P<0.001）。肾小球硬化(P=0.036),IFTA(P=0.008)是ESRD的预测因子。感染（P=0.02），呼吸衰竭（P<0.001），心力衰竭（P=0.004）是死亡的危险因素。
结论：II型RPGN是最常见的。感染是最常见的次要结果。少尿症,肾小球硬化,演示时需要进行血液透析，IFTA和TLO形成是ESRD的预测因子。呼吸衰竭,心力衰竭和感染是死亡率的重要预测因子.
方法：我们回顾性评估了101例18岁以上的成人患者，这些患者根据肾活检诊断为RPGN，这些患者的肾小球中新月≥50%。排除在肾活检中具有<50％的新月的患者以及拒绝同意肾活检的患者。我们根据免疫组织化学将患者分为3组；I型，II型和III型。然后，取决于肾脏的损失，我们将他们分为ESRD和非ESRD组。检索临床病史和体格检查。此外,24小时尿蛋白，尿液分析,肾功能试验,血清白蛋白,全血细胞计数,抗核抗体,抗双链DNA抗体,检查ANCA抗体和血清补体水平。每位患者都接受了肾脏活检以进行免疫组织化学和光学显微镜检查。新月肾小球的百分比，硬化肾小球的数量，三级淋巴器官（TLO），中性粒细胞浸润，毛细血管内或系膜细胞增多，分析了间质纤维化伴肾小管萎缩(IFTA)。主要结果（缓解，评估ESRD和死亡率）和次要结局。
结果：II型是RPGN的最常见原因（47.5％），其次是III型（32.7％）和I型（19.8％）。32例（31.7%）在随访期间死亡，而60例患者（59.4%）发展为ESRD。41例患者（40.6%），出现缓解。少尿症,血清肌酐,与非ESRD组相比,ESRD组出现时对HD的需求显著增加(各P<0.001)。系膜增生,IFTA,TLO形成，与非ESRD组相比，ESRD组的硬化性肾小球和纤维新月也显着增加（每个P<0.001）。肾小球硬化(P=0.036),IFTA(P=0.008)是ESRD的预测因子。感染（P=0.02），呼吸衰竭（P<0.001），心力衰竭（P=0.004）是死亡的危险因素。
结论：II型RPGN是最常见的。感染是最常见的次要结果。少尿症,肾小球硬化,演示时需要进行血液透析，IFTA和TLO形成是ESRD的预测因子。呼吸衰竭,心力衰竭和感染是死亡率的重要预测因子.
