Kadoya Hiroyuki, Namba Tomoko, Matsuda Jun, Yamamoto Takeshi, Takeji Masanori, Yamauchi Atsushi
Division of Nephrology, Osaka Rosai Hospital, Osaka, Japan.
Nihon Jinzo Gakkai Shi. 2010;52(5):590-4.
AL amyloidosis is the most common form of systemic amyloidosis. Although kidney biopsy often is the method by which the disease is identified, small amounts of amyloid in kidney biopsy specimens may be missed on routine examination unless specifically investigated. We present here a previously healthy 60-year-oldmissed on routine examination unless specifically investigated. We present here a previously healthy 60-year-old man who developed nephrotic syndrome. His first renal biopsy showed minimal change nephrotic syndromeman who developed nephrotic syndrome. His first renal biopsy showed minimal change nephrotic syndromesuggesteda subtle depost ommon formsystemicamyloidfibrilhediminationof an early lesion of renal amyloido-s (MCNS). Proteinuria remained refractory to immunosuppressive treatments. Six months later, a repeat renal biopsy clearly showed AL amyloidosis. Re-examination of the first biopsy in the light of the final diagnosis again suggested a subtle deposition of amyloid fibrils. The discrimination of an early lesion of renal amyloidosis with MCNS may often be difficult. It is necessary to maintain a high level of alertness for amyloidosis especially in aged patients with nephrotic syndrome and to consider a repeat biopsy in steroid-resistant cases.
AL淀粉样变性是系统性淀粉样变性最常见的形式。尽管肾活检常常是确诊该疾病的方法，但除非进行专门检查，否则肾活检标本中的少量淀粉样物质在常规检查时可能会被遗漏。我们在此报告一名60岁的既往健康男性，他出现了肾病综合征。其首次肾活检显示为微小病变型肾病综合征（MCNS）。蛋白尿对免疫抑制治疗无效。6个月后，再次肾活检明确显示为AL淀粉样变性。根据最终诊断重新检查首次活检结果，再次提示有淀粉样纤维的细微沉积。区分早期肾淀粉样变性病变与MCNS往往很困难。对于淀粉样变性必须保持高度警惕，尤其是在患有肾病综合征的老年患者中，并且对于激素抵抗的病例要考虑再次活检。
