Esteve V, Almirall J, Ponz E, García N, Ribera L, Larrosa M, Andreu X, García M
Servicio de Nefrología, Corporació Sanitaria Parc Taulí, Institut Universitari Parc Taulí (UAB), Sabadell.
Nefrologia. 2006;26(2):212-7.
Systemic amyloidosis is a disease resulting from extracellular deposition of fibrillar protein in various organs. Main systemic amyloidosis are: primary (AL) and Secondary (AA). The kidney is usually involved, conferring and adverse prognosis. In the last decade there has been a change in the aetiology of AA amyloidosis.
To analyse the incidence of AL and AA amyloidosis in our current population as well as the aetiology of AA amyloidosis. To describe clinical outcomes, renal involvement and survival.
We performed a descriptive analysis of all cases of amyloidosis diagnosed from 1992 to 2004 in our hospital. Diagnosis was assessed on histological criteria: positivity Congo Red stain. Clinical data, renal involvement, dialysis treatment and survival were analysed.
76 cases, 44 women, mean age 70.7 +/- 12. Types: 55 AA (72%), 21 AL (28%) systemic amyloidosis. AA aetiology was: 66% rheumatic disorders, 28% infectious disease, 6% others. Incidence for AL was 4.6 and for AA 12.2 cases/million. Renal involvement was present in 75% at diagnosis (69% Creatinine clearance < 60 ml/min, 37% urinary protein > 3 g/24 hours). 21 cases (28%) progressed to renal disease stage V in the 8.1 +/- 9.8 months follow up period, and 14 cases started dialysis treatment (10 HD, 4 CAPD). In 7 cases (33%) dialysis was not indicated due to their poor clinical condition, short life expectancy and bad quality of life. Mean global survival at diagnosis was 55% and 40% at 12 and 24 months (AL 58% and 19%; AA 55% and 44%). Mean survival from the start of dialysis was 30% and 5% at 12 and 24 months.
Although amyloidosis has a low incidence in our population, the kidney is usually involved. Rheumatological disorders are the principal aetiology of AA amyloidosis. Long term survival is poor, specially for AL.
系统性淀粉样变性是一种由于纤维状蛋白在各器官细胞外沉积而导致的疾病。主要的系统性淀粉样变性类型有：原发性（AL）和继发性（AA）。肾脏通常会受累，从而导致不良预后。在过去十年中，AA淀粉样变性的病因发生了变化。
分析我们当前人群中AL和AA淀粉样变性的发病率以及AA淀粉样变性的病因。描述临床结局、肾脏受累情况和生存率。
我们对1992年至2004年在我院诊断的所有淀粉样变性病例进行了描述性分析。根据组织学标准进行诊断：刚果红染色阳性。分析临床数据、肾脏受累情况、透析治疗和生存率。
共76例，其中女性44例，平均年龄70.7±12岁。类型：55例AA（72%），21例AL（28%）系统性淀粉样变性。AA的病因如下：66%为风湿性疾病，28%为传染病，6%为其他。AL的发病率为每百万4.6例，AA为每百万12.2例。诊断时75%的患者存在肾脏受累（69%的患者肌酐清除率<60 ml/分钟，37%的患者尿蛋白>3 g/24小时）。在8.1±9.8个月的随访期内，21例（28%）进展至肾脏疾病V期，14例开始透析治疗（10例血液透析，4例持续性非卧床腹膜透析）。7例（33%）因临床状况差、预期寿命短和生活质量差而未进行透析。诊断时的总体平均生存率在12个月和24个月时分别为55%和40%（AL分别为58%和19%；AA分别为55%和44%）。开始透析后的平均生存率在12个月和24个月时分别为30%和5%。
尽管淀粉样变性在我们的人群中发病率较低，但肾脏通常会受累。风湿性疾病是AA淀粉样变性的主要病因。长期生存率较低，尤其是AL型。
