Persistence of renal function in a patient diagnosed with concurrent amyloid amyloidosis and immunoglobulin light chain amyloidosis.
Persistence of renal function in a patient diagnosed with concurrent amyloid amyloidosis and immunoglobulin light chain amyloidosis.
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被诊断患有并发淀粉样变性和免疫球蛋白轻链淀粉样变性的患者的肾功能持续存在。
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发表时间：
2016
期刊：
影响因子：
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通讯作者：
H. Nakashima
中科院分区：
文献类型：
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作者：
R. Watanabe;Kenji Ito;T. Yasuno;Yasuhiro Abe;A. Hamauchi;T. Yasunaga;Y. Sasatomi;S. Hisano;Takao Saito;H. Nakashima
本病例描述了一位68岁的女性，在55岁时表现出最初的蛋白尿。随后，在57岁时，通过肾活检检查诊断为淀粉样淀粉样变性（a a）和免疫球蛋白（Ig）轻链淀粉样变性（AL）的混合型淀粉样变性。单克隆副蛋白血症同时被鉴定并诊断为不确定意义单克隆γ病（MGUS）。开始联合美法兰和强的松龙（MP）治疗。在65岁时，发现尿蛋白浓度升高和血压升高时，给予抗高血压药物。由于骨髓活检检查未发现MGUS状态的变化，因此停止MP治疗。然而，尿蛋白浓度升高，并在66岁时再次进行肾活检。这显示了早期诊断的AA和AL的混合型淀粉样变，但在此期间肾小球内AL淀粉样沉积增加。此后继续维持生命治疗，但肾病综合征和肾功能障碍进展迅速。终末期肾衰竭沉积很少见于同一个体。虽然一般认为淀粉样变会导致肾功能迅速下降，但该患者的肾功能维持了13年。这可能归因于以下因素：1)AA淀粉样变的潜在病因尚不清楚，2)目前缺乏任何慢性炎症的证据，3)MGUS是AL淀粉样变的原因。这与MP治疗一起，可能减缓了通常与AL淀粉样变相关的病理性衰退。
This case describes a 68-year-old woman exhibiting initial proteinuria at age 55. Subsequently, at age 57, a mixed-type of amyloidosis consisting of amyloid amyloidosis (A A) and immunoglobulin (Ig) light chain amyloidosis (AL) was diagnosed by a renal biopsy examination. Monoclonal paraproteinemia was concurrently identified and diagnosed as monoclonal gammopathy of undeterminate significance (MGUS). Combined melphalan and prednisolone (MP) therapy was initiated. At age 65, anti-hypertensive drugs were administered upon finding an increased urine protein concentration and elevated blood pressure. Because there was no change in the state of MGUS detected by a bone marrow biopsy examination, MP therapy was discontinued. However, the urinary protein concentration increased, and a renal biopsy was performed again at age 66. This revealed a mixed-type amyloidosis of AA and AL, as diagnosed earlier, but AL amyloid deposition in the glomeruli had increased during the intervening period. Life-preserving treatment was continued thereafter, but nephrotic syndrome and renal dysfunction progressed rapidly. End-stage renal failure deposition is rarely seen in the same individual. Although amyloidosis is generally thought to cause a rapid decline in renal function, the patient's renal function was maintained for 13 years. This could be attributed to the following factors : l)the underlying etiology of the AA amyloidosis, which was not clear, 2)a lack of any current evidence of chronic inflammation, and 3) MGUS as the cause of AL amyloidosis. This, together with MP therapy, may have slowed down the pathological decline normally associated with AL amyloidosis.