Establishment of diagnostic criteria and disease severity in Crow-Fukase syndrome

2018 Fiscal Year Final Research Report

• Project/Area Number: 16K09692
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Chiba University
• Principal Investigator: Misawa Sonoko 千葉大学, 医学部附属病院, 准教授 (30375753)
• Project Period (FY): 2016-04-01 – 2019-03-31
• Keywords: クロウ-深瀬症候群 / POEMS症候群 / 血管内皮増殖因子 / 形質細胞

Outline of Final Research Achievements

Crow-Fukase (POEMS) syndrome is a rare cause of demyelinating neuropathy based on plasma cell dyscrasia. The prognosis of Crow-Fukase syndrome has been improved by application of treatment for myeloma. However, appropriate diagnostic criteria and disease severity are essential to establish efficient therapeutic strategies. Therefore, we have suggested a new diagnostic criteria, which was statistically validated and had high diagnostic accuracy. In addition, we analyzed prevalence rate and current treatment status, and prognosis of Crow-Fukase syndrome in Japan based on the national survey conducted in 2015. We are now considering disease severity based on our database, the national survey data, and national registry database.

Free Research Field

神経内科

Academic Significance and Societal Importance of the Research Achievements

クロウ-深瀬（POEMS）症候群は全国の患者数が400例未満の超稀少疾患である。患者数が少ないが故に、保険適用のある治療がなく、治療方針も標準化されていない。本研究により、現時点の我が国におけるクロウ・深瀬症候群の罹病者数、疾患プロファイル、治療の実態が明確になった。本症候群は骨髄腫の治療が奏功する可能性が高く、診断基準の整備、治療の標準化により、予後の改善が大いに期待される。本研究成果により、クロウ・深瀬症候群の今後の予後改善の基盤が築かれた。