Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan
Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan
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DOI：
10.1111/j.1440-1843.2006.00810.x
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发表时间：
2006-01-01
期刊：
影响因子：
6.9
通讯作者：
Sakatani, M
中科院分区：
文献类型：
--
作者：
Inoue, Y;Nakata, K;Sakatani, M
特发性肺泡蛋白沉积症(IPAP)是一种罕见的疾病，其特征是肺泡内有过多的脂蛋白物质。本文对日本肺泡蛋白沉积症患者的全国流行病学资料进行了中期分析，并对IPAP血清标志物的作用进行了分析(I)显示了166例日本IPAP患者的全国人口学资料。男女之比为1：2，确诊时平均年龄51.14岁(15~79岁)。共有30%的IPAP患者的临床病程较差。总共有30%的患者接受了全肺灌洗疗法(WWL)。在WLL下，患者在短期内显著改善，但接受WLL的患者中有40%再次恶化。难治性PAP的治疗需要粒细胞巨噬细胞集落刺激因子(GM-CSF)等新的治疗策略。(2)血清KL-6、癌胚抗原、表面活性蛋白D和A、LDH与疾病严重程度的相关性提示它们有可能作为疾病标志物。相反，血清抗GM-CSF抗体与病情严重程度无关，而是诊断WAR的特异性标志物。联合检测血清标志物对IPAP患者的诊断和治疗有很大帮助。
Idiopathic pulmonary alveolar proteinosis (IPAP) is a rare disease characterized by excessive amounts of lipoproteinaceous material in the alveolus. This report presents an interim analysis of nationwide epidemiological data from Japanese patients with pulmonary alveolar proteinosis, and the roles of serum markers for IPAP (i) The nationwide demographic data from 166 Japanese patients with IPAP are shown. The female to male ratio was 1:2, and the average age was 51 14 years old (age range: 15-79 years) at registration or diagnosis. A total of 30% of patients with IPAP have a poor clinical course. In total, 30% of patients were treated with whole lung lavage therapy (WWL). UnderWLL, the patients significantly improved in the short term, but 40% of the patients who underwent WLL worsened again. A new strategy such as granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy for intractable PAP is required. (ii) The correlation of serum KL-6, carcinoembryonic antigen, surfactant proteins D and A, and LDH with disease severity suggests their potential as disease markers. In contrast, serum anti-GM-CSF antibody did not correlate with disease severity, but is a specific marker for the diagnosis of WAR The combined measurements of the serum markers may well prove very useful for both the diagnosis and the management of IPAP patients.