Clinical Course of GM-CSF Autoantibody Negative Pulmonary Alveolar Proteinosis (NAPAP): Efficacy of Lavage Therapy.
Clinical Course of GM-CSF Autoantibody Negative Pulmonary Alveolar Proteinosis (NAPAP): Efficacy of Lavage Therapy.
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GM-CSF 自身抗体阴性肺泡蛋白沉积症 (NAPAP) 的临床过程：灌洗疗法的疗效。
DOI：
10.1164/ajrccm-conference.2009.179.1_meetingabstracts.a3035
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发表时间：
2009
期刊：
影响因子：
--
通讯作者：
H. Goto
中科院分区：
文献类型：
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作者：
H. Ishii;K. Nakata;Y. Inoue;R. Tazawa;Y. Tsuchihashi;H. Goto
由于NAPAP的罕见性，其预后和治疗效果的信息很少。然而，由于近年来BAL和VATS等诊断方法的发展，此类病例的报告越来越多。< Methods>收集1999年至2008年360例病理诊断病例。其中NAPAP 38例（继发性PAP 34例，未分型PAP 4例），对其预后进行回顾性分析。对于分析，疾病严重程度评分定义如下：评分1; SpO 2 95%<和PS 0 × 2，2; SpO 2 95%<和PS 3 × 4，3; SpO 2 90 × 94%和PS 0 × 2，4; SpO 2 90 × 94%和PS 3 × 4，5; SpO 2 <90%，6;通气或死亡。< Result>2年总生存率为62.1%。血液系统疾病病例的情况更糟（n= 29：53.6%）。14例患者接受了肺灌洗治疗（全肺灌洗：n= 8，节段性支气管肺泡灌洗：n= 6），治疗前疾病严重程度评分为4.0±1.5，治疗后为4.5±1.9（p= 0.25）。其中，只有两个病例得到改善。而4例接受移植（脐带血移植、外周血干细胞移植、骨髓移植、肺移植）的患者，移植后均进展满意。< Conclusion>NAPAP合并血液系统疾病的预后很差。虽然肺灌洗治疗被认为是标准治疗，但其疗效存在争议。
Little information is available for the prognosis and efficacy of therapy in patients with NAPAP because of rarity of the disease. However, the cases have been increasingly reported owing to recent development of the diagnostic methods such as BAL and VATS.< Methods> Three hundred sixty pathologically diagnosed cases were enrolled since 1999 to 2008. Of those, 38 cases were NAPAP (secondary PAP: n= 34, unclassified PAP: n= 4) and were investigated on the prognosis retrospectively. For analyses, disease severity score was defined as follows: score 1; SpO2 95%< and PS 0χ2, 2; SpO2 95%< and PS 3χ4, 3; SpO2 90χ94% and PS 0χ2, 4; SpO2 90χ94% and PS 3χ4, 5; SpO2< 90%, 6; ventilation or death.< Result> Two years overall survival rate: was 62.1%. It was worse in cases with hematological disorders (n= 29: 53.6%). Fourteen cases underwent lung lavage therapy (whole lung lavage: n= 8, segmental bronchoalveolar lavage: n= 6) with the disease severity score of 4.0±1.5 before the therapy, whereas it was 4.5±1.9 after the therapy (p= 0.25). Of those, only two cases improved. In contrast, all of 4 cases who underwent transplantation (cord blood transplantation, peripheral blood stem cell transplantation, bone marrow transplantation, lung transplantation) were making satisfactory progress after transplantation.< Conclusion> Prognosis of NAPAP with hematological disorders is very poor. Although lung lavage therapy is known as the standard therapy, the efficacy is controversial.