Rysavá R, Merta M, Tesar V, Zabka J, Spicka I, Stejskalová A, Vernerová Z
I. interní klinika 1. LF UK a VFN, Praha.
Cas Lek Cesk. 1998 Feb 23;137(4):107-9.
Renal amyloid involvement results either from primary or secondary amyloidosis. Extent of amyloid tissue deposition in kidneys and clinical course depends not only on the type of basic process but reflects also time of diagnosis and possibility to influence the basic process.
We analyzed laboratory and clinical data of patients with bioptically proven renal amyloidosis. We found renal amyloidosis in 27 patients from an overall number of 750 renal biopsies (RB) performed in our department (i.e. 3.6%). AA amyloidosis was diagnosed in 16 pts, AL amyloidosis in 11 pts. About 50% of patients had laboratory signs of nephrotic syndrome, all patients had various degree of proteinuria. Impaired renal function were found in more than 50% of patients, in 6 of them we had to start renal replacement therapy. 8 pts died. Complications of severe nephrotic syndrome were the causes of death in majority of cases. We have started investigation of some amyloid precursors and cytokines in patients with AA and AL amyloidosis. We compared the results with group of patients with vasculitis. We investigated plasma and urinary levels of SAA (serum AA) and soluble receptor for interleukin 2 (sIL-2R).
Clinical features and laboratory findings in our patients with renal amyloidosis approximately are in accordance with literary data. Plasmatic level of SAA was increased not only in the group of patients with AA amyloidosis, but also in the group of vasculitis. Urinary sIL-2R was significantly increased in patients with AA amyloidosis in comparison with healthy controls.
肾脏淀粉样变可由原发性或继发性淀粉样变性引起。淀粉样组织在肾脏中的沉积程度及临床病程不仅取决于基础病变的类型,还反映了诊断时间以及影响基础病变的可能性。
我们分析了经活检证实的肾淀粉样变性患者的实验室和临床数据。在我们科室进行的750例肾活检(RB)中,共发现27例肾淀粉样变性患者(即3.6%)。诊断为AA型淀粉样变性16例,AL型淀粉样变性11例。约50%的患者有肾病综合征的实验室指标,所有患者均有不同程度的蛋白尿。超过50%的患者存在肾功能损害,其中6例患者不得不开始肾脏替代治疗。8例患者死亡。大多数病例中,严重肾病综合征的并发症是死亡原因。我们已开始对AA型和AL型淀粉样变性患者的一些淀粉样前体和细胞因子进行研究。我们将结果与血管炎患者组进行了比较。我们检测了血浆和尿液中SAA(血清淀粉样蛋白A)和白细胞介素2可溶性受体(sIL-2R)的水平。
我们的肾淀粉样变性患者的临床特征和实验室检查结果大致与文献数据相符。SAA的血浆水平不仅在AA型淀粉样变性患者组中升高,在血管炎患者组中也升高。与健康对照组相比,AA型淀粉样变性患者的尿sIL-2R显著升高。