International clinical research for secondary pulmonary alveolar proteinosis complicated with myelodysplastic syndrome

2016 Fiscal Year Final Research Report

• Project/Area Number: 26305028
• Research Category: Grant-in-Aid for Scientific Research (B)
• Allocation Type: Partial Multi-year Fund
• Section: 海外学術
• Research Field: Respiratory organ internal medicine
• Research Institution: Kyorin University
• Principal Investigator: Ishii Haruyuki 杏林大学, 医学部, 准教授 (30406970)
• Co-Investigator(Kenkyū-buntansha): 中田 光 新潟大学, 医歯学総合病院, 教授 (80207802) ; 井上 義一 独立行政法人国立病院機構(近畿中央胸部疾患センター臨床研究センター), その他部局等, その他 (90240895) ; 田澤 立之 新潟大学, 医歯学総合病院, 准教授 (70301041) ; 皿谷 健 杏林大学, 医学部, 助教 (40549185)
• Project Period (FY): 2014-04-01 – 2017-03-31
• Keywords: 肺胞蛋白症 / 骨髄異形成症候群 / 呼吸器内科 / 血液内科 / 内科 / ステロイド

Outline of Final Research Achievements

We investigated the clinical research for secondary pulmonary alveolar proteinosis complicated with myelodysplastic syndrome. There was little clinical information for this disease because SPAP complicated with MDS was extremely rare lung disease. We demonstrated the following outcomes, based on the results from our study. 1)Prevalence of SPAP complicated with MDS in Japan was significant higher than it in Europe. 2)The prognosis of SPAP complicated with MDS was very poor after diagnosis of SPAP(MST: 15 months). 3)It is the most important risk factor for the prognosis of SPAP was the use of steroid therapy at diagnosis of SPAP, and its cox hazard ratio for the death was 2.43. These outcomes will contribute to the medical treatment and new study for SPAP complicated with MDS. Moreover it is good outcome to be able to constitite the international SPAP network.

Free Research Field

びまん性肺疾患