Abstract：
OBJECTIVE: We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still\'s disease (AOSD).
METHODS: We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed.
RESULTS: The median age at the diagnosis of renal issues was 37, with a higher prevalence among females (58.1%). Among the cases, 28 experienced renal problems after being diagnosed with AOSD, 12 had simultaneous diagnoses of renal issues and AOSD, and in 4 cases, renal problems appeared before AOSD diagnosis. Out of the 44 cases, 36 underwent renal biopsy, revealing various pathology findings including AA amyloidosis (25%), collapsing glomerulopathy (11.4%), thrombotic microangiopathy (TMA) (11.4%), IgA nephropathy (9.1%), minimal change disease (6.8%), and others. Some cases were clinically diagnosed with TMA, proximal tubular dysfunction, or macrophage activation syndrome-related acute kidney injury. Treatment approaches varied, but glucocorticoids were commonly used. Renal involvement was associated with increased mortality and morbidity, with 6 out of 44 patients passing away, 4 progressing to end-stage renal disease (ESRD), and data on 2 cases\' outcomes not available.
CONCLUSIONS: Renal manifestations in AOSD are diverse but rarely studied owing to the rarity of the disease. Studies with larger data would be essential to study further on the pathogenesis and implications.
METHODS: We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed.
RESULTS: The median age at the diagnosis of renal issues was 37, with a higher prevalence among females (58.1%). Among the cases, 28 experienced renal problems after being diagnosed with AOSD, 12 had simultaneous diagnoses of renal issues and AOSD, and in 4 cases, renal problems appeared before AOSD diagnosis. Out of the 44 cases, 36 underwent renal biopsy, revealing various pathology findings including AA amyloidosis (25%), collapsing glomerulopathy (11.4%), thrombotic microangiopathy (TMA) (11.4%), IgA nephropathy (9.1%), minimal change disease (6.8%), and others. Some cases were clinically diagnosed with TMA, proximal tubular dysfunction, or macrophage activation syndrome-related acute kidney injury. Treatment approaches varied, but glucocorticoids were commonly used. Renal involvement was associated with increased mortality and morbidity, with 6 out of 44 patients passing away, 4 progressing to end-stage renal disease (ESRD), and data on 2 cases\' outcomes not available.
CONCLUSIONS: Renal manifestations in AOSD are diverse but rarely studied owing to the rarity of the disease. Studies with larger data would be essential to study further on the pathogenesis and implications.
摘要：
目的：我们旨在回顾有关临床表现的文献，肾脏病理学，治疗,成人发作的斯蒂尔病（AOSD）的肾脏表现和预后。
方法：我们使用PRISMA指南进行系统评价，包括从开始到2023年9月15日的所有英文原创文章，涉及任何形式的AOSD和肾脏受累。患者人口统计数据，诊断标准,临床表现,肾脏病理学，包括透析在内的治疗方法，结果,收集死亡原因并进行分析。
结果：诊断为肾脏疾病的中位年龄为37岁，女性患病率较高（58.1%）。在案件中，28人在被诊断为AOSD后出现肾脏问题，12人同时诊断肾脏问题和AOSD，在4个案例中，肾脏问题出现在AOSD诊断前.在44个案例中，36人接受了肾活检，揭示各种病理结果，包括AA淀粉样变性（25％），塌陷性肾小球病(11.4%)，血栓性微血管病（TMA）（11.4%），IgA肾病（9.1%），微小变化疾病(6.8%)，和其他人。一些病例临床诊断为TMA，近端肾小管功能障碍,或巨噬细胞活化综合征相关的急性肾损伤。治疗方法多种多样，但糖皮质激素是常用的。肾脏受累与死亡率和发病率增加相关，44名患者中有6人去世，4进展为终末期肾病（ESRD），和2例结果的数据不可用。
结论：AOSD的肾脏表现是多样的，但由于该疾病的稀有性，很少进行研究。具有更大数据的研究对于进一步研究发病机理和意义至关重要。
方法：我们使用PRISMA指南进行系统评价，包括从开始到2023年9月15日的所有英文原创文章，涉及任何形式的AOSD和肾脏受累。患者人口统计数据，诊断标准,临床表现,肾脏病理学，包括透析在内的治疗方法，结果,收集死亡原因并进行分析。
结果：诊断为肾脏疾病的中位年龄为37岁，女性患病率较高（58.1%）。在案件中，28人在被诊断为AOSD后出现肾脏问题，12人同时诊断肾脏问题和AOSD，在4个案例中，肾脏问题出现在AOSD诊断前.在44个案例中，36人接受了肾活检，揭示各种病理结果，包括AA淀粉样变性（25％），塌陷性肾小球病(11.4%)，血栓性微血管病（TMA）（11.4%），IgA肾病（9.1%），微小变化疾病(6.8%)，和其他人。一些病例临床诊断为TMA，近端肾小管功能障碍,或巨噬细胞活化综合征相关的急性肾损伤。治疗方法多种多样，但糖皮质激素是常用的。肾脏受累与死亡率和发病率增加相关，44名患者中有6人去世，4进展为终末期肾病（ESRD），和2例结果的数据不可用。
结论：AOSD的肾脏表现是多样的，但由于该疾病的稀有性，很少进行研究。具有更大数据的研究对于进一步研究发病机理和意义至关重要。
