Magy-Bertrand N, Dupond J-L, Mauny F, Dupond A-S, Duchene F, Gil H, Kantelip B
Department of Internal Medicine, University Hospital Jean Minjoz, Besançon, France.
Clin Exp Rheumatol. 2008 Nov-Dec;26(6):1074-8.
There is a lack of epidemiological information concerning amyloidosis, particularly in France. We started a 3-year prospective study (AMPYRO) to analyze the epidemiological features of amyloidosis in the eastern part of France.
From 2003 to 2005, all patients with a tissue sample showing amyloid deposits, were included in this study. Immunohistochemistry using anti-P component, anti-SAA, anti-light chains immunoglobulins and anti-transthyretin was applied for each tissue sample. For each patient, past and present medical histories along with biological features were recorded.
Seventy-six patients with amyloid were identified over 3 years. The age-standardized incidence rate of amyloidosis was estimated at 14 cases per million person-years. The final entire population included in the AMYPRO study was composed of 66 patients with a mean age of 71.7+/-11.5 years old. The amyloid typing after clinical, biological and immunohistochemistry revealed senile amyloid in 40 cases (60.6%), AL amyloid in 13 (19.7%) and AA amyloid in 9 (13.6%). Neither clinical nor biological features differed significantly between the transthyretin-positive and transthyretin-negative populations.
Regarding only tissue samples, senile amyloid was the most prominent amyloid type identified. Therefore, the clinician needs to be aware that in most of the amyloid cases identified on the pathologic examination there is no need for additional examination unless there are clinical or biological signs of a primary or secondary amyloidosis.
关于淀粉样变性病,尤其是在法国,缺乏流行病学信息。我们启动了一项为期3年的前瞻性研究(AMPYRO),以分析法国东部淀粉样变性病的流行病学特征。
2003年至2005年,所有组织样本显示有淀粉样蛋白沉积的患者均纳入本研究。对每个组织样本应用抗P成分、抗SAA、抗轻链免疫球蛋白和抗转甲状腺素蛋白的免疫组织化学方法。记录每位患者的既往和当前病史以及生物学特征。
3年期间共识别出76例淀粉样变性病患者。淀粉样变性病的年龄标准化发病率估计为每百万人年14例。AMYPRO研究最终纳入的全部人群由66例患者组成,平均年龄为71.7±11.5岁。经临床、生物学和免疫组织化学检查后的淀粉样蛋白分型显示,40例(60.6%)为老年性淀粉样变性,13例(19.7%)为AL型淀粉样变性,9例(13.6%)为AA型淀粉样变性。转甲状腺素蛋白阳性和阴性人群在临床和生物学特征上均无显著差异。
仅就组织样本而言,老年性淀粉样变性是最主要的淀粉样蛋白类型。因此,临床医生需要意识到,在病理检查中发现的大多数淀粉样变性病例,除非有原发性或继发性淀粉样变性的临床或生物学体征,否则无需进一步检查。