The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients.
The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients.
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全身性淀粉样变性的临床特征和结果：231例中国患者的队列。
DOI：
10.1093/ckj/sfu117
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发表时间：
2015-02
影响因子：
4.6
通讯作者：
Liu Z
中科院分区：
文献类型：
--
作者：
Huang X;Wang Q;Jiang S;Chen W;Zeng C;Liu Z
关于中国系统性免疫球蛋白轻链（AL）淀粉样变性患者的临床特征和结局的数据很少。本研究的目的是揭示一个大型中国AL淀粉样变性患者队列的临床表现和疾病进展的危险因素。对2003年至2011年金陵医院淀粉样变性登记处的患者进行了研究。从首次就诊至死亡或直至末次可用的临床随访，收集临床和实验室信息。分析患者的生存率和肾脏结局，并评估临床参数与生存率之间的关系。本研究共入组231例患者，所有研究的患者均有肾脏受累。153例（66.2%）为男性，诊断时的中位年龄为56岁。轻链λ型198例（85.7%）。147例（63.6%）患者表现为肾病综合征（NS），25%的患者在诊断时有肾功能不全。κ型淀粉样变性患者肝脏受累和NS更常见，肾损害更严重。中位生存期36.3个月，1、2、3、5年累计生存率分别为67、53、48、35%。多因素考克斯分析显示，年龄、肝脏受累和心脏受累可显著影响这些患者的生存率。患者保持无透析的中位时间为50个月。1年、2年、3年和5年时保持无透析的患者百分比分别为78%、69%、62%和37%。多因素考克斯分析显示血肌酐和低血压是肾功能衰竭的重要危险因素。λ型是中国人AL淀粉样变性的主要类型。AL淀粉样变性患者的生存率很差。危险因素包括心脏和肝脏受累、低血压和肾功能损害。诊断时高血清肌酐水平和低血压与肾脏预后不良相关。
Few data are available on the clinical features and outcomes of Chinese patients with systemic immunoglobulin light-chain (AL) amyloidosis. The aim of this study is to reveal the clinical picture and risk factors of disease progression in a large cohort of Chinese patients with AL amyloidosis. Patients in the Jinling Hospital amyloidosis registry from 2003 to 2011 were studied. The clinical and laboratory information were collected from first presentation to death or until the last available clinical follow-up. The patients' survival and renal outcomes were analyzed, and the relationships between the clinical parameters and survival were also assessed. A total of 231 patients were enrolled in this study, all the patients studied had renal involvement. One hundred and fifty-three (66.2%) were male, and the median age at diagnosis was 56 years. A total of 198 (85.7%) cases had light-chain λ-type. One hundred and forty-seven (63.6%) cases presented as nephrotic syndrome (NS), and 25% of patients had renal insufficiency at diagnosis. Liver involvement and NS appeared to be more common in patients of κ-type amyloidosis, and renal impairment is more severe in κ-type amyloidosis. The median survival time of all patients was 36.3 months, and the 1-, 2-, 3- and 5-year cumulative survival rates were 67, 53, 48 and 35%, respectively. Multivariate COX analysis showed that age, hepatic involvement and heart involvement can significantly influence survival in these patients. The median time that patients remained dialysis free was 50 months. The percentage of patients that remained dialysis free at 1, 2, 3 and 5 years were 78, 69, 62 and 37%, respectively. Multivariate COX analysis showed that serum creatinine and hypotension were the important risk factors of renal failure. λ-Type is the most dominant type of AL amyloidosis in Chinese patients. The survival of patients with AL amyloidosis is poor. The risk factors included heart and hepatic involvement, hypotension and impairment of renal function. The high serum creatinine level and hypotension at diagnosis are associated with poor renal outcome.