࢚ࣅࢹࣥࢫᇶ࡙࠸ࡓ⚄⤒චᝈࡢ᪩ᮇデ᩿࣭㔜ᗘศ㢮࣭⒪ࣝࢦࣜࢬ࣒ࡢ☜❧㸸
POEMS ೃ⩌࣭୰ᯡᮎᲈ㐃ྜ⬺㧊࣭ࢨࢵࢡೃ⩌ࡢデ᩿ᇶ‽⟇ᐃ
⌜ ဨ ᱓ཎ⪽
1)ඹྠ◊✲⪅ ୕⃝ᅬᏊ
1)ࠊูᗓ⨾ዉᏊ
1)ࠊ㛵ཱྀ⦕
1)ࠊబ⸨Ὀ᠇
2)ࠊྜྷⰋ₶୍
3)ࠊ
♽∗Ụඖ
4)ࠊᯇ┿
5)◊✲せ᪨
POEMS ೃ⩌ࠊ୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡣࠊᅜෆ᥎ᐃᝈ⪅ᩘࡀ 100㹼300 ྡࡢ⛥
ᑡ㞴ᛶ⚄⤒ᝈ࡛࠶ࡾࠊデ᩿ᇶ‽ࠊ⮬↛Ṕࠊ⒪ἲ࡞ࡣ☜❧ࡋ࡚࠸࡞࠸ࠋᨻ⟇◊✲⌜࡛࠶ࡿࠕ࢚
ࣅࢹࣥࢫᇶ࡙࠸ࡓ⚄⤒චᝈࡢ᪩ᮇデ᩿࣭㔜ᗘศ㢮࣭⒪ࣝࢦࣜࢬ࣒ࡢ☜❧◊✲⌜ࠖࡢᴗ
ࡋ࡚ࡇࡢ3ᝈࡘ࠸࡚デ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞ࡗࡓࠋPOEMSೃ⩌ࡘ࠸࡚ࡣ⮬㦂98࠾ࡼࡧ
ᝈࢥࣥࢺ࣮ࣟࣝ63㸦៏ᛶ⅖ᛶ⬺㧊ᛶከⓎࢽ࣮ࣗࣟࣃࢳ࣮32ࠊከⓎᛶ㦵㧊⭘࣭AL࣑ࣟ
ࢻ࣮ࢩࢫ 30 㸧ࡢ⮫ᗋ≧ࠊ᳨ᰝᡤぢࢆヲ⣽ศᯒࡋࠊసᡂࡋࡓデ᩿ᇶ‽ࡢឤᗘࠊ≉␗ᗘࢆ᳨ウࡍ
ࡿࡇࡼࡾࠊࡑࡢጇᙜᛶࢆ᳨ドࡋࡓࠋከⓎࢽ࣮ࣗࣟࣃࢳ࣮࣭M⺮ⓑ⾑࣭⾑Ύ⾑⟶ෆ⓶ቑṪᅉᏊቑ ຍࡢ 3 㡯┠ࢆᇶ‽ࡋ࡚᪂ࡓ⟇ᐃࡋࡓデ᩿ᇶ‽ࡢឤᗘࡣ 100㸣ࠊ≉␗ᗘࡣ98㸣࡛࠶ࡾࠊᚋࡢ デ⒪᥎ዡ࡛ࡁࡿࠋ୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡘ࠸࡚ࡣᬻᐃデ᩿ᇶ‽ࢆᥦၐࡋࡓࠋ
A㸬◊✲┠ⓗ
POEMS ೃ⩌㸦ࢡ࣭ࣟ࢘῝℩ೃ⩌㸧ࠊ୰ᯡ
ᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡣࠊ࠸ࡎࢀ
ࡶᅜෆ᥎ᐃᝈ⪅ᩘࡀ 100㹼350 ྡࡉࢀࡿ⛥ᑡ
⚄⤒ᝈ࡛࠶ࡿࠋࡑࡢ⛥ᑡᛶᨾᝈᴫᛕࠊデ
᩿ᇶ‽ࠊ⮬↛Ṕ࣭ணᚋࠊ⒪ἲ࡞ࡢ☜❧ࡣ࡞
ࡉࢀ࡚࠾ࡽࡎࠊࡇࢀࡽࢆ᪩ᛴᩚഛࡍࡿࡇࡀ ồࡵࡽࢀ࡚࠸ࡿࠋ
POEMS ೃ⩌ࡣᙧ㉁⣽⬊ࣔࣀࢡ࣮ࣟࢼࣝቑ
Ṫ⾑⟶ෆ⓶ቑṪᅉᏊ㸦VEGF㸧ࡢ㐣⏘⏕ࢆᇶ
┙ከⓎࢽ࣮ࣗࣟࣃࢳ࣮࣭⮚ჾ⭘࣭ෆศἪ␗
ᖖ࣭⓶␗ᖖ➼ࡢከᙬ࡞≧ࢆ࿊ࡍࡿ㌟ᛶ
ᝈ࡛࠶ࡿࠋࡇࢀࡲ࡛」ᩘࡢ㢮ఝࡢデ᩿ᇶ‽ࡀᥦ ၐࡉࢀ࡚࠸ࡿࡀࠊឤᗘ࣭≉␗ᗘࢆ᳨ウࡋࡓሗ࿌
ࡣ࡞࠸ࠋ㐣ཤሗ࿌ࡉࢀࡓྛデ᩿ᇶ‽ࡣ࠸ࡎࢀ
ࡶᇶ‽ᑠᇶ‽ࡢ⤌ࡳྜࢃࡏࡼࡾデ᩿
1) ༓ⴥᏛᏛ㝔་Ꮫ◊✲㝔࣭⚄⤒ෆ⛉Ꮫ
2)༓ⴥᏛ་Ꮫ㒊㝃ᒓ㝔࣭⮫ᗋヨ㦂㒊
3) ᕞᏛᏛ㝔་Ꮫ◊✲㝔࣭⬻⚄⤒◊✲
タ࣭⚄⤒ෆ⛉
4) ྡྂᒇᏛᏛ㝔་Ꮫ⣔◊✲㝔◊✲⛉࣭
⚄⤒ෆ⛉Ꮫ
5) 㔠ἑ་⛉Ꮫ་Ꮫ㒊࣭⚄⤒ෆ⛉Ꮫ
⤫ィᏛⓗ࡞ゎᯒࡣ⾜࡞ࢃࢀ࡚࠸࡞࠸ࠋࡲࡓࠊ
⒪⤒㐣ࢆྵࡵ࡚⥲ྜⓗ☜ᐃデ᩿ࡋࡓ⮬㦂
ࢆgold standard㞟ᅋࡋ࡚ࠊឤᗘ࣭≉␗ᗘࡢ㧗
࠸デ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞࠺ࠋ
୰ᯡᮎᲈ㐃ྜ⬺㧊ࡣࠊ୰ᯡ⚄⤒ᮎᲈ⚄⤒
ࡢ᪉⬺㧊ࢆ⏕ࡌࡿ⮬ᕫචᝈ࡛࠶ࡾࠊᢠ
neurofascin ᢠయࡢୖ᪼ࡀሗ࿌ࡉࢀ࡚࠸ࡿࠋ⌧
Ⅼ࡛ࡣ୰ᯡᮎᲈ㐃ྜ⬺㧊ㄪᰝ◊✲⌜ࡼࡿ
デ᩿ᇶ‽㸦ᖹᡂ24ᖺ㸧ࡀ࠶ࡾࠊࡇࢀࢆඖデ᩿
ᇶ‽ࡢ⟇ᐃࢆ⾜࡞࠺ࠋ
ࢨࢵࢡೃ⩌ࡣᮎᲈ⚄⤒㍈⣴ࡢ㐣⯆ዧ
ࡼࡾ➽②ᨥ࣭◳┤ࢆࡁࡓࡍᝈ࡛࠶ࡾࠊࡇࢀ
ࡽࡢᡤぢࢆ୰᰾ࡋࡓデ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞
࠺ࠋ
B㸬◊✲᪉ἲ
㸯㸬POEMSೃ⩌ࡢᝈᴫせデ᩿ᇶ‽
ᝈᴫせࡘ࠸࡚ࡣᩥ⊩᳨⣴ཬࡧ⮬㦂98ࡢ
⮫ᗋሗࡢศᯒࡽసᡂࡋࡓࠋデ᩿ᇶ‽ࡣྠ
ೃ⩌ᝈ⪅98ࠊᝈᑐ↷62 㹙៏ᛶ⅖ᛶ⬺
㧊ᛶከⓎ⚄⤒⅖㸦CIDP㸧࣭ከⓎᛶ㦵㧊⭘࣭༢ࢡ
࣮ࣟࣥᛶ࣐࢞ࣥࢢࣟࣈࣜࣥ⾑࣭AL࣑ࣟࢻ
࣮ࢩࢫ㹛ࢆᑐ㇟ࡋࡓࠋ⌧ᅾᥦၐࡉࢀ࡚࠸ࡿ 3 ࡘࡢデ᩿ᇶ‽㸦Dispenzeri.20143㸧ࠊKuwabara et al. 20121)ࠊMisawa and Kuwabara., 20134㸧㸧
࢚ࣅࢹࣥࢫᇶ࡙࠸ࡓ⚄⤒චᝈࡢ᪩ᮇデ᩿࣭㔜ᗘศ㢮࣭⒪ࣝࢦࣜࢬ࣒ࡢ☜❧㸸
POEMS ೃ⩌࣭୰ᯡᮎᲈ㐃ྜ⬺㧊࣭ࢨࢵࢡೃ⩌ࡢデ᩿ᇶ‽⟇ᐃ
⌜ ဨ ᱓ཎ⪽
1)ඹྠ◊✲⪅ ୕⃝ᅬᏊ
1)ࠊูᗓ⨾ዉᏊ
1)ࠊ㛵ཱྀ⦕
1)ࠊబ⸨Ὀ᠇
2)ࠊྜྷⰋ₶୍
3)ࠊ
♽∗Ụඖ
4)ࠊᯇ┿
5)◊✲せ᪨
POEMS ೃ⩌ࠊ୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡣࠊᅜෆ᥎ᐃᝈ⪅ᩘࡀ 100㹼300 ྡࡢ⛥
ᑡ㞴ᛶ⚄⤒ᝈ࡛࠶ࡾࠊデ᩿ᇶ‽ࠊ⮬↛Ṕࠊ⒪ἲ࡞ࡣ☜❧ࡋ࡚࠸࡞࠸ࠋᨻ⟇◊✲⌜࡛࠶ࡿࠕ࢚
ࣅࢹࣥࢫᇶ࡙࠸ࡓ⚄⤒චᝈࡢ᪩ᮇデ᩿࣭㔜ᗘศ㢮࣭⒪ࣝࢦࣜࢬ࣒ࡢ☜❧◊✲⌜ࠖࡢᴗ
ࡋ࡚ࡇࡢ3ᝈࡘ࠸࡚デ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞ࡗࡓࠋPOEMSೃ⩌ࡘ࠸࡚ࡣ⮬㦂98࠾ࡼࡧ
ᝈࢥࣥࢺ࣮ࣟࣝ63㸦៏ᛶ⅖ᛶ⬺㧊ᛶከⓎࢽ࣮ࣗࣟࣃࢳ࣮32ࠊከⓎᛶ㦵㧊⭘࣭AL࣑ࣟ
ࢻ࣮ࢩࢫ30 㸧ࡢ⮫ᗋ≧ࠊ᳨ᰝᡤぢࢆヲ⣽ศᯒࡋࠊసᡂࡋࡓデ᩿ᇶ‽ࡢឤᗘࠊ≉␗ᗘࢆ᳨ウࡍ
ࡿࡇࡼࡾࠊࡑࡢጇᙜᛶࢆ᳨ドࡋࡓࠋከⓎࢽ࣮ࣗࣟࣃࢳ࣮࣭M⺮ⓑ⾑࣭⾑Ύ⾑⟶ෆ⓶ቑṪᅉᏊቑ ຍࡢ3 㡯┠ࢆᇶ‽ࡋ࡚᪂ࡓ⟇ᐃࡋࡓデ᩿ᇶ‽ࡢឤᗘࡣ 100㸣ࠊ≉␗ᗘࡣ98㸣࡛࠶ࡾࠊᚋࡢ デ⒪᥎ዡ࡛ࡁࡿࠋ୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡘ࠸࡚ࡣᬻᐃデ᩿ᇶ‽ࢆᥦၐࡋࡓࠋ
A㸬◊✲┠ⓗ
POEMS ೃ⩌㸦ࢡ࣭ࣟ࢘῝℩ೃ⩌㸧ࠊ୰ᯡ
ᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡣࠊ࠸ࡎࢀ
ࡶᅜෆ᥎ᐃᝈ⪅ᩘࡀ 100㹼350 ྡࡉࢀࡿ⛥ᑡ
⚄⤒ᝈ࡛࠶ࡿࠋࡑࡢ⛥ᑡᛶᨾᝈᴫᛕࠊデ
᩿ᇶ‽ࠊ⮬↛Ṕ࣭ணᚋࠊ⒪ἲ࡞ࡢ☜❧ࡣ࡞
ࡉࢀ࡚࠾ࡽࡎࠊࡇࢀࡽࢆ᪩ᛴᩚഛࡍࡿࡇࡀ ồࡵࡽࢀ࡚࠸ࡿࠋ
POEMS ೃ⩌ࡣᙧ㉁⣽⬊ࣔࣀࢡ࣮ࣟࢼࣝቑ
Ṫ⾑⟶ෆ⓶ቑṪᅉᏊ㸦VEGF㸧ࡢ㐣⏘⏕ࢆᇶ
┙ከⓎࢽ࣮ࣗࣟࣃࢳ࣮࣭⮚ჾ⭘࣭ෆศἪ␗
ᖖ࣭⓶␗ᖖ➼ࡢከᙬ࡞≧ࢆ࿊ࡍࡿ㌟ᛶ
ᝈ࡛࠶ࡿࠋࡇࢀࡲ࡛」ᩘࡢ㢮ఝࡢデ᩿ᇶ‽ࡀᥦ ၐࡉࢀ࡚࠸ࡿࡀࠊឤᗘ࣭≉␗ᗘࢆ᳨ウࡋࡓሗ࿌
ࡣ࡞࠸ࠋ㐣ཤሗ࿌ࡉࢀࡓྛデ᩿ᇶ‽ࡣ࠸ࡎࢀ
ࡶᇶ‽ᑠᇶ‽ࡢ⤌ࡳྜࢃࡏࡼࡾデ᩿
1) ༓ⴥᏛᏛ㝔་Ꮫ◊✲㝔࣭⚄⤒ෆ⛉Ꮫ
2)༓ⴥᏛ་Ꮫ㒊㝃ᒓ㝔࣭⮫ᗋヨ㦂㒊
3) ᕞᏛᏛ㝔་Ꮫ◊✲㝔࣭⬻⚄⤒◊✲
タ࣭⚄⤒ෆ⛉
4) ྡྂᒇᏛᏛ㝔་Ꮫ⣔◊✲㝔◊✲⛉࣭
⚄⤒ෆ⛉Ꮫ
5) 㔠ἑ་⛉Ꮫ་Ꮫ㒊࣭⚄⤒ෆ⛉Ꮫ
⤫ィᏛⓗ࡞ゎᯒࡣ⾜࡞ࢃࢀ࡚࠸࡞࠸ࠋࡲࡓࠊ
⒪⤒㐣ࢆྵࡵ࡚⥲ྜⓗ☜ᐃデ᩿ࡋࡓ⮬㦂
ࢆgold standard㞟ᅋࡋ࡚ࠊឤᗘ࣭≉␗ᗘࡢ㧗
࠸デ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞࠺ࠋ
୰ᯡᮎᲈ㐃ྜ⬺㧊ࡣࠊ୰ᯡ⚄⤒ᮎᲈ⚄⤒
ࡢ᪉⬺㧊ࢆ⏕ࡌࡿ⮬ᕫචᝈ࡛࠶ࡾࠊᢠ
neurofascin ᢠయࡢୖ᪼ࡀሗ࿌ࡉࢀ࡚࠸ࡿࠋ⌧
Ⅼ࡛ࡣ୰ᯡᮎᲈ㐃ྜ⬺㧊ㄪᰝ◊✲⌜ࡼࡿ
デ᩿ᇶ‽㸦ᖹᡂ24ᖺ㸧ࡀ࠶ࡾࠊࡇࢀࢆඖデ᩿
ᇶ‽ࡢ⟇ᐃࢆ⾜࡞࠺ࠋ
ࢨࢵࢡೃ⩌ࡣᮎᲈ⚄⤒㍈⣴ࡢ㐣⯆ዧ
ࡼࡾ➽②ᨥ࣭◳┤ࢆࡁࡓࡍᝈ࡛࠶ࡾࠊࡇࢀ
ࡽࡢᡤぢࢆ୰᰾ࡋࡓデ᩿ᇶ‽ࡢ⟇ᐃࢆ⾜࡞
࠺ࠋ
B㸬◊✲᪉ἲ
㸯㸬POEMSೃ⩌ࡢᝈᴫせデ᩿ᇶ‽
ᝈᴫせࡘ࠸࡚ࡣᩥ⊩᳨⣴ཬࡧ⮬㦂98ࡢ
⮫ᗋሗࡢศᯒࡽసᡂࡋࡓࠋデ᩿ᇶ‽ࡣྠ
ೃ⩌ᝈ⪅98ࠊᝈᑐ↷62㹙៏ᛶ⅖ᛶ⬺
㧊ᛶከⓎ⚄⤒⅖㸦CIDP㸧࣭ከⓎᛶ㦵㧊⭘࣭༢ࢡ
࣮ࣟࣥᛶ࣐࢞ࣥࢢࣟࣈࣜࣥ⾑࣭AL࣑ࣟࢻ
࣮ࢩࢫ㹛ࢆᑐ㇟ࡋࡓࠋ⌧ᅾᥦၐࡉࢀ࡚࠸ࡿ 3 ࡘࡢデ᩿ᇶ‽㸦Dispenzeri.20143㸧ࠊKuwabara et al. 20121)ࠊMisawa and Kuwabara., 20134㸧㸧
ᇶ‽࠾ࡼࡧᑠᇶ‽㡯┠ࢆᢳฟࡋࠊࣟࢪࢫࢸࢵ
ࢡᅇᖐศᯒ࡛ኚᩘ㑅ᢥࢆᐇࡋ ROC ᭤⥺ࡽ
ឤᗘཬࡧ≉␗ᗘࡀ᭱࡞ࡿࡶࡢࢆ㑅ࢇࡔࠋస ᡂࡋࡓデ᩿ᇶ‽ࡢឤᗘ࣭≉␗ᗘࢆ᳨ウࡋࡓࠋ
㸰㸬୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡢ
ᝈᴫせࠊデ᩿ᇶ‽
ᩥ⊩ࢆ㞟ࡍࡿࡶࠊ㐣ཤࡢ◊✲⌜ࡼ
ࡿሗ࿌5)⪃ࡋ࡚సᡂࡋࡓࠋ
⌮㠃ࡢ㓄៖
ᮏ◊✲㝿ࡋ࡚ࡣࠊ༓ⴥᏛᏛ㝔་Ꮫ◊✲㝔
࠾ࡼࡧ་Ꮫ㒊㝃ᒓ㝔ࡢ⌮つᐃࢆ㑂Ᏺࡋ࡚⾜
ࡗࡓࠋ⾑Ύ᳨యࡢ⏝㛵ࡋ࡚ࡣᝈ⪅ࡽࡣ
ࣥࣇ࢛࣮࣒ࢻ࣭ࢥࣥࢭࣥࢺࢆᚓࡓࠋಶேࡢሗ ࡣỴࡋ࡚⾲ฟࡿࡇࡀ࡞࠸ࡼ࠺㸪ࡲࡓࣉࣛ
ࣂࢩ࣮ࡢಖㆤࡘ࠸࡚ࡶࠊ༑ศ㓄៖ࡋࡓࠋ 㑇ఏሗ㛵ࡍࡿྲྀࡾᢅ࠸ࡢヱᙜࡣ࡞ࡗࡓࠋ C㸬⤖ᯝ
㸯㸬POEMSೃ⩌ࡢデ᩿ᇶ‽
⾲㸯᪂つ⟇ᐃࡋࡓࢡ࣭ࣟ࢘ࣇ࢝ࢭೃ⩌
ࡢデ᩿ᇶ‽ࢆ♧ࡍࠋᇶ‽ࡋ࡚ከⓎࢽ࣮ࣗࣟ
ࣃࢳ࣮㸦ᚲ㡲㸧ࠊM⺮ⓑࠊ⾑Ύ⾑⟶ෆ⓶ቑṪᅉᏊ 㸦VEGF㸧㸦ᚲ㡲㸧␗ᖖ㧗್ࡢ 3 㡯┠ࢆタᐃࡋࠊ ࠕprobableࠖࡢ㝵ᒙデ᩿ࡣᑡ࡞ࡃࡶ2㡯┠ࢆ
‶ࡓࡍࡇࡋࡓࠋPOEMS ೃ⩌ 98࠾ࡅ
ࡿ㝧ᛶ⋡ࡣࡑࢀࡒࢀ 100㸣ࠊ92㸣ࠊ100㸣࡛࠶ࡗ ࡓࠋࡍ࡞ࢃࡕ8㸣ࡢ࠾࠸࡚ M⺮ⓑࡣ㝜ᛶ
࡛࠶ࡗࡓࠋᑠᇶ‽ࡋ࡚ࠕ⓶␗ᖖ㸦๛ẟࠊⰍ
⣲ỿ╔ࠊ⾑⟶⭘㸧ࠊ⾑ᑠᯈቑከࠊ㦵◳ᛶኚࠊ ᾋ⭘࣭⬚⭡Ỉࡢ4 㡯┠ࡢ࠺ࡕ1㡯┠ࢆ‶ࡓࡍࠖ
タᐃࡋࡓࠋࡇࡢデ᩿ᇶ‽ࡢឤᗘࡣ100%ࠊ≉␗
ᗘࡣ98㸣࡛࠶ࡗࡓࠋ
᪤ሗ࿌㸦Dispenzieri 2014ཬࡧKuwabara et al., 2012㸧ࡢデ᩿ᇶ‽࡛ࡣ༢ࢡ࣮ࣟࣥᛶࡢᙧ㉁⣽⬊
ቑṪࢆᚲ㡲㡯┠ࡋ࡚࠸ࡿࡓࡵデ᩿ឤᗘࡣ 92㸣ࡲࡗࡓࠋචᅛᐃἲ࡛M⺮ⓑࡀ㝜ᛶ
࡛㦵㧊␗ᖖ࣭ᙧ㉁⣽⬊⭘ࡶ᳨ฟࡉࢀ࡞࠸ᮏೃ
⩌ᝈ⪅ࡀ 8㸣Ꮡᅾࡍࡿࡇࡀ♧ࡉࢀࡓࠋࡲࡓ CIDPᝈ⪅࡛M⺮ⓑ㝧ᛶࡀ21㸣ࠊVEGFୖ᪼ࡀ 12㸣Ꮡᅾࡋࡓࡀࠊᑠᇶ‽ࡢ㐺⏝ࡼࡾࠊࡇࢀ
ࡽࡢᝈ⪅ࡣ㝖እࡉࢀࡓࠋ
㸰㸬୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡢ
ᬻᐃデ᩿ᇶ‽
୰ᯡᮎᲈ㐃ྜ⬺㧊ࡢデ᩿ᇶ‽ࢆ⾲㸰♧ࡍࠋ ᚲ㡲㡯┠ࡋ࡚(1)୰ᯡ⚄⤒ࡢ⬺㧊ࡀ MRI ࠶ࡿ
࠸ࡣㄏⓎ㟁࡛♧ࡉࢀࡿࡇࠊ(2)ᮎᲈ⚄⤒ࡢ⬺
㧊ᡤぢࡣ⚄⤒ఏᑟ᳨ᰝ࡛㟁Ẽデ᩿ᇶ‽ࢆ‶ࡓࡍ
ࡶࡢ࡛࠶ࡿࡇࠊ(3)୰ᯡ࣭ᮎᲈ⚄⤒ࢆࡍከ
ᝈࡀ㝖እࡉࢀࡿࡇࡋࡓࠋࢨࢵࢡೃ⩌
ࡢデ᩿ᇶ‽ࢆ⾲㸱♧ࡍࠋࡇࢀࡽࡢデ᩿ᇶ‽
ࡘ࠸࡚ࡣᚋࡢ✚ࡼࡿ᳨ドࡀᚲせ࡛࠶
ࡿᛮࢃࢀࡓࠋ
㻰䠊⪃ᐹ㻌
ࢡ࣭ࣟ࢘῝℩ೃ⩌ࡢデ᩿ᇶ‽ࡣࡇࢀࡲ࡛
」ᩘࡢࡶࡢࡀᥦၐࡉࢀ࡚࠸ࡿࡀࠊࡑࡢឤᗘ࣭≉
␗ᗘ㛵ࡍࡿሗ࿌ࡣ࡞ࡉࢀ࡚࠸࡞࠸ࠋᅇ᳨ウ ࡋ ࡓ 3 ࡘ ࡢ デ ᩿ ᇶ ‽ ࡢ ࠺ ࡕ ࡛ ࠊ Dispenzeri.20143㸧ࠊKuwabara et al. 20121)ࠊ࡛
ࡣM⺮ⓑ⾑ࡀᚲ㡲㡯┠ࡉࢀ࡚࠾ࡾࠊMisawa and Kuwabara., 20134㸧࡛ࡣ⾑ΎVEGFୖ᪼ࡀ ᚲ㡲㡯┠࡞ࡗ࡚࠸ࡿⅬࡀデ᩿ᇶ‽ࡢጇᙜᛶࢆ
᳨ドࡍࡿୖ࡛㔜せ࡞࣏ࣥࢺ࡞ࡿࡇࡀ♧ࡉ
ࢀࡓࠋ⣙10㸣ࡢᝈ⪅࠾࠸࡚M⺮ⓑࡀ᳨ฟࡉࢀ
࡞ࡗࡓࡇࡽ๓⪅ࡢឤᗘࡣ90㸣࡛࠶ࡗࡓࠋ ᚑࡗ࡚M⺮ⓑࢆᚲ㡲㡯┠ࡍࡿࡇࡣၥ㢟ࡀ
࠶ࡿ⪃࠼ࡽࢀࠊ᪂つデ᩿ᇶ‽࡛ࡣᚲ㡲ࡣࡋ
࡞ࡗࡓࠋ
୍᪉⾑ΎVEGF㧗್ࡣ100㸣࡛ㄆࡵࡽࢀࡓࡇ
ࡽ᪂つᇶ‽ࡢឤᗘࡣ 100㸣࡛࠶ࡾࠊࡋࡶ
ᝈᑐ↷㸦៏ᛶ⅖ᛶ⬺㧊ᛶከⓎࢽ࣮ࣗࣟࣃࢳ
࣮࣭ከⓎᛶ㦵㧊⭘࣭AL࣑ࣟࢻ࣮ࢩࢫ㸧ᑐ ࡍࡿ≉␗ᗘࡣ98㸣࡛࠶ࡾࠊࡇࡢᇶ‽ࡢጇᙜᛶࡀ
♧ࡉࢀࠊ᭱ࡶ㐺ษ࡞ࡶࡢ࡛࠶ࡿࡇࡀ⤖ㄽ࡛ࡁ
ࡿᛮࢃࢀࡿࠋࡇࢀࡽࡢ⤖ᯝࢆᇶࠊᙜ◊✲⌜
࡛ࡣ⾲㸯ࡢデ᩿ᇶ‽ࢆ᥎ዡࡋࠊࡉࡽ๓ྥࡁ◊
✲ࡼࡾ᳨ドࢆ㐍ࡵࡿࠋ
୰ᯡᮎᲈ㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡢデ
᩿ᇶ‽ࡘ࠸࡚ࡣጇᙜᛶࢆ᳨ドࡍࡿᝈ⪅㞟ᅋࡀ
ࡲࡔᏑᅾࡏࡎࠊᬻᐃⓗ࡞ࡶࡢ࡞ࡿࠋ୰ᯡᮎᲈ 㐃ྜ⬺㧊࡛ࡣⓎᵝᘧ㸦ᛴᛶ㹼៏ᛶ㸧ࢆၥࢃ
࡞࠸ࡶࡢࡋ࡚࠸ࡿࡓࡵࠊᛴᛶ㓟ᅾᛶ⬻⬨㧊⅖
⬺㧊ᆺࢠ࣭ࣛࣥࣂ࣮ࣞೃ⩌ࡢྜేࠊ࠶ࡿ࠸
ࡣከⓎᛶ◳៏ᛶ⅖ᛶ⬺㧊ᛶከⓎࢽ࣮ࣗ
ࣟࣃࢳ࣮ࡢྜేࢆྵࡴࡇ࡞ࡾࠊࡇࢀࡽࡢ
ែࡢ␗ྠࡣᚋ᳨ドࡉࢀࡿࡁ࡛࠶ࡿᛮࢃ
ࢀࡿࠋࡲࡓ㝖እᝈࡘ࠸࡚ࡶࡉࡽᗈࡃタᐃ ࡀᚲせ࡞ࡿྍ⬟ᛶࡀ࠶ࡾࠊᚋࢆ㞟✚ࡋ
᳨࡚ウࢆ㐍ࡵࡿࡁ࡛࠶ࡿ⪃࠼ࡽࢀࡿࠋ
㻱䠊⤖ㄽ㻌
ࢡ࣭ࣟ࢘῝℩ೃ⩌୰ᯡᮎᲈ㐃ྜ⬺㧊ࡢデ
᩿ᇶ‽ࡢ⟇ᐃࢆヨࡳࡓࠋࢡ࣭ࣟ࢘῝℩ೃ⩌ࡢ デ᩿ᇶ‽ࡋ࡚ᙜ◊✲⌜ࡋ࡚᥎ዡ࡛ࡁࡿᇶ‽
ࢆ⟇ᐃࡋࠊࡑࡢ᭷⏝ᛶࡀ᳨ドࡉࢀࡓࠋ୰ᯡᮎᲈ 㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡢデ᩿ᇶ‽ࡘ
࠸࡚ࡣᚋࡢ㞟✚ࢆ⾜࠸ࡉࡽ᳨ウࡋ࡚࠸
ࡃᚲせࡀ࠶ࡿࡶࡢᛮࢃࢀࡓࠋ
ᩥ⊩㻌
1. Y Kuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev. 2012 Jun 13;6:CD006828.
2. Kawamura N, Yamasaki R, Yonekawa T, Matsushita T, Kusunoki S, Nagayama S, Fukuda Y, Ogata H, Matsuse D, Murai H, Kira J. Anti-neurofascin antibody in patients with combined central and peripheral demyelination. Neurology.
2013 Aug 20;81(8):714-22.
3. Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014 Feb;89(2):214-23.
4. Misawa S, Kuwabara S. Polyneuropathy, organomegaly, endocrinopathy,
monoclonal gammopathy and skin changes (Crow–Fukase) syndrome:
Diagnostic criteria and treatment perspectives. Clin Exp Neuroimmunol 2013;4:318-325.
5. ୰ᯡᮎᲈ㐃ྜ⬺㧊㸦ᖹᡂᖺᗘ㸧 http://www.nanbyou.or.jp/entry/3309 㹄.◊✲Ⓨ⾲㸦ᖹᡂ26-28ᖺᗘ㸧
㸦㸯㸧ᅜෆ
ཱྀ㢌Ⓨ⾲ 㸯㸶௳
ཎⴭㄽᩥࡼࡿⓎ⾲ 㸶௳
ࡑࢀ௨እ(ࣞࣅ࣮ࣗ➼)ࡼࡿⓎ⾲ 㸰௳
ࡑࡢ࠺ࡕ࡞ࡶࡢ Ⓨ⾲ㄽᩥ
1. ᱓ཎ⪽㸬POEMS ೃ⩌㸬EBM ᇶ࡙ࡃ
⬻⚄⤒ᝈࡢᇶᮏ⏝ᣦ㔪㸬࣓ࢹ࢝ࣝࣞࣅࣗ
࣮♫㸬2016ᖺ㸸662-664㸬
㸦㸰㸧ᾏእⓎ⾲
ཱྀ㢌Ⓨ⾲ 㸳௳
ཎⴭㄽᩥࡼࡿⓎ⾲ 㸯㸶௳
ࡑࢀ௨እ(ࣞࣅ࣮ࣗ➼)ࡼࡿⓎ⾲ 㸱௳
ࡑࡢ࠺ࡕ࡞ࡶࡢ Ⓨ⾲ㄽᩥ
1. Misawa S, Sato Y, Katayama K, Nagashima K, Aoyagi R, Sekiguchi Y, Sobue G, Koike H, Yabe I, Sasaki H, Watanabe O, Takashima H, Nishizawa M, Kawachi I, Kusunoki S, Mitsui Y, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S; Japanese POEMS Syndrome for Thalidomide (J-POST) Trial Study Group.. Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol.
2016 Oct;15(11):1129-37.
2. Misawa S, Sato Y, Katayama K, Hanaoka H, Sawai S, Beppu M, Nomura F, Shibuya K, Sekiguchi Y, Iwai Y, Watanabe K, Amino H, Ohwada C, Takeuchi M, Sakaida E, Nakaseko C, Kuwabara S. Vascular endothelial growth factor as a predictive marker for POEMS syndrome treatment response: retrospective cohort study. BMJ Open. 2015 Nov 11;5(11):e009157.
㻳䠊▱ⓗᡤ᭷ᶒ䛾ฟ㢪䞉Ⓩ㘓≧ἣ㻌 㻌 㻌
≉チྲྀᚓ࣭ᐇ⏝᪂Ⓩ㘓㸸ヱᙜ࡞ࡋ
᳨࡚ウࢆ㐍ࡵࡿࡁ࡛࠶ࡿ⪃࠼ࡽࢀࡿࠋ
㻱䠊⤖ㄽ㻌
ࢡ࣭ࣟ࢘῝℩ೃ⩌୰ᯡᮎᲈ㐃ྜ⬺㧊ࡢデ
᩿ᇶ‽ࡢ⟇ᐃࢆヨࡳࡓࠋࢡ࣭ࣟ࢘῝℩ೃ⩌ࡢ デ᩿ᇶ‽ࡋ࡚ᙜ◊✲⌜ࡋ࡚᥎ዡ࡛ࡁࡿᇶ‽
ࢆ⟇ᐃࡋࠊࡑࡢ᭷⏝ᛶࡀ᳨ドࡉࢀࡓࠋ୰ᯡᮎᲈ 㐃ྜ⬺㧊ࠊࢨࢵࢡೃ⩌ࡢデ᩿ᇶ‽ࡘ
࠸࡚ࡣᚋࡢ㞟✚ࢆ⾜࠸ࡉࡽ᳨ウࡋ࡚࠸
ࡃᚲせࡀ࠶ࡿࡶࡢᛮࢃࢀࡓࠋ
ᩥ⊩㻌
1. Y Kuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev. 2012 Jun 13;6:CD006828.
2. Kawamura N, Yamasaki R, Yonekawa T, Matsushita T, Kusunoki S, Nagayama S, Fukuda Y, Ogata H, Matsuse D, Murai H, Kira J. Anti-neurofascin antibody in patients with combined central and peripheral demyelination. Neurology.
2013 Aug 20;81(8):714-22.
3. Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014 Feb;89(2):214-23.
4. Misawa S, Kuwabara S. Polyneuropathy, organomegaly, endocrinopathy,
monoclonal gammopathy and skin changes (Crow–Fukase) syndrome:
Diagnostic criteria and treatment perspectives. Clin Exp Neuroimmunol 2013;4:318-325.
5. ୰ᯡᮎᲈ㐃ྜ⬺㧊㸦ᖹᡂᖺᗘ㸧 http://www.nanbyou.or.jp/entry/3309 㹄.◊✲Ⓨ⾲㸦ᖹᡂ26-28ᖺᗘ㸧
㸦㸯㸧ᅜෆ
ཱྀ㢌Ⓨ⾲ 㸯㸶௳
ཎⴭㄽᩥࡼࡿⓎ⾲ 㸶௳
ࡑࢀ௨እ(ࣞࣅ࣮ࣗ➼)ࡼࡿⓎ⾲ 㸰௳
ࡑࡢ࠺ࡕ࡞ࡶࡢ Ⓨ⾲ㄽᩥ
1. ᱓ཎ⪽㸬POEMS ೃ⩌㸬EBM ᇶ࡙ࡃ
⬻⚄⤒ᝈࡢᇶᮏ⏝ᣦ㔪㸬࣓ࢹ࢝ࣝࣞࣅࣗ
࣮♫㸬2016ᖺ㸸662-664㸬
㸦㸰㸧ᾏእⓎ⾲
ཱྀ㢌Ⓨ⾲ 㸳௳
ཎⴭㄽᩥࡼࡿⓎ⾲ 㸯㸶௳
ࡑࢀ௨እ(ࣞࣅ࣮ࣗ➼)ࡼࡿⓎ⾲ 㸱௳
ࡑࡢ࠺ࡕ࡞ࡶࡢ Ⓨ⾲ㄽᩥ
1. Misawa S, Sato Y, Katayama K, Nagashima K, Aoyagi R, Sekiguchi Y, Sobue G, Koike H, Yabe I, Sasaki H, Watanabe O, Takashima H, Nishizawa M, Kawachi I, Kusunoki S, Mitsui Y, Kikuchi S, Nakashima I, Ikeda S, Kohara N, Kanda T, Kira J, Hanaoka H, Kuwabara S; Japanese POEMS Syndrome for Thalidomide (J-POST) Trial Study Group.. Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol.
2016 Oct;15(11):1129-37.
2. Misawa S, Sato Y, Katayama K, Hanaoka H, Sawai S, Beppu M, Nomura F, Shibuya K, Sekiguchi Y, Iwai Y, Watanabe K, Amino H, Ohwada C, Takeuchi M, Sakaida E, Nakaseko C, Kuwabara S. Vascular endothelial growth factor as a predictive marker for POEMS syndrome treatment response: retrospective cohort study. BMJ Open. 2015 Nov 11;5(11):e009157.
㻳䠊▱ⓗᡤ᭷ᶒ䛾ฟ㢪䞉Ⓩ㘓≧ἣ㻌 㻌 㻌
≉チྲྀᚓ࣭ᐇ⏝᪂Ⓩ㘓㸸ヱᙜ࡞ࡋ
⾲㸯㸬ࢡ࣭ࣟ࢘῝℩ೃ⩌ࡢデ᩿ᇶ‽
ᇶ‽ 㸸
㸯㸬ከⓎࢽ࣮ࣗࣟࣃࢳ࣮
㸰㸬⾑ΎVEGFୖ᪼ 㸦1000 pg/ml ௨ୖ㸧
㸱㸬M⺮ⓑ 㸦⾑Ύࡲࡓࡣᒀ୰M⺮ⓑ㝧ᛶ [චᅛᐃἲࡼࡾ☜ㄆ] 㸧
ᑠᇶ‽ 㸸
㸯㸬㦵◳ᛶኚ 㸰㸬ᾋ⭘ࠊ⬚⭡Ỉ 㸱㸬㦵◳ᛶኚ 㸲㸬⾑ᑠᯈቑከ
ͤࡓࡔࡋࠊ⏥≧⭢ᶵ⬟␗ᖖࠊ⮅⮚ᶵ⬟␗ᖖࡘ࠸࡚ࡣ᭷⋡ࡀ㧗࠸ࡓࡵ༢⊂ࡢ␗ᖖ࡛ࡣᑠᇶ‽ࡢ1 㡯
┠ࡋ࡚᥇⏝ࡋ࡞࠸ࠋ
-Definite ᇶ‽ࢆ3 㡯┠ࡶ‶ࡓࡋࡘᑠᇶ‽ࢆ2 㡯┠௨ୖ‶ࡓࡍ
-Probable ᇶ‽ࡢ࠺ࡕᮎᲈ⚄⤒㞀ᐖ⾑Ύ VEGFୖ᪼ࢆ‶ࡓࡋࠊࡘᑠᇶ‽1 㡯┠௨ୖ‶ࡓࡍ
-Possible ᇶ‽ࡢ࠺ࡕᮎᲈ⚄⤒㞀ᐖࢆ‶ࡓࡋࠊࡘᑠᇶ‽ࢆ1 㡯┠௨ୖ‶ࡓࡍ
⾲㸰㸬୰ᯡᮎᲈ㐃ྜ⬺㧊ࡢデ᩿ᇶ‽
ᚲ㡲㡯┠
ձ ⬻࣭⬨㧊T2㧗ಙྕMRIᕢ㸬࠶ࡿ࠸ࡣど⚄⤒MRIࡲࡓࡣVEP࡛␗ᖖࡀㄆࡵࡽࢀࡿ
ղ ᮎᲈ⚄⤒ఏᑟ᳨ᰝ࡛⬺㧊ᡤぢࡀㄆࡵࡽࢀࡿ
ճ ཎᅉᝈࡀ᫂ࡽ࡛࡞࠸㸬
⚄⤒࣎ࣞࣜឤᰁ࡞ࡢឤᰁ㸪⚄⤒ࢧࣝࢥࢻ࣮ࢩࢫࡸ⚄⤒࣮࣋ࢳ࢙ࢵࢺ㸪㛵⠇࣐ࣜ࢘ࢳ࡞
⅖ᛶ࣭⮬ᕫචᛶ㸪ࣅࢱ࣑ࣥḞஈࡸ៏ᛶࣝࢥ࣮ࣝ୰ẘ࡞ࡢ௦ㅰᛶᝈ㸪ⓑ㉁ࢪࢫࢺࣟࣇ࣮
࡞ࡢ㑇ఏᏊᝈࡢࡼ࠺㸪୰ᯡ⚄⤒ᮎᲈ⚄⤒ࡢ୧⪅ࢆࡍཎᅉࡢ᫂ࡽ࡞ࡶࡢࡣྵࡵ࡞࠸㸬
*Ⓨࡢᵝᘧࡣၥࢃ࡞࠸㸦ᛴᛶࠊளᛴᛶࠊ៏ᛶ㸧
**⚄⤒ఏᑟ᳨ᰝࡢ㐺᱁ᇶ‽㸸ṇ୰⚄⤒㸪ᑻ㦵⚄⤒㸪⬯㦵⚄⤒ࡢ࠺ࡕ㸪ᑡ࡞ࡃࡶ 2 ࡘࡢ⚄⤒࠾࠸
࡚㸪⬺㧊ࢆ♧၀ࡍࡿ㟁Ẽデ᩿ᇶ‽ 㸦EFNS/PNSᇶ‽㸧ࢆ⏝࠸ࡿ
ձ㹼ճࢆ‶ࡓࡍࡶࡢࢆᮏࡍࡿ
⾲㸱㸬ࢨࢵࢡೃ⩌ࡢデ᩿ᇶ‽
A㸬せ≧࣭ᡤぢ
㸯㸬╧╀ࡶᣢ⥆ࡍࡿᅄ⫥࣭యᖿࡢ➽②ᨥࡲࡓࡣ➽◳┤㸦ᚲ㡲㸧 㸰㸬ᮎᲈ⚄⤒㐣⯆ዧࢆ♧ࡍ➽㟁ᅗᡤぢ
㸦Myokymic discharge, neuromyotonic discharge㸧 㸱㸬⾑ΎᢠVGKC」ྜయᢠయ㝧ᛶ 㸦>72pM㸧
㸲㸬⭈⓶㉁ࢫࢸࣟࢻࠊ⾑₢⒪ἲ࡞ࡢච⒪࡛ᨵၿ B㸬ᨭᣢ≧࣭ᡤぢ
㸯㸬Ⓨờ㐣ከ
㸰㸬ᅄ⫥ࡢ③ࡳ࣭␗ᖖឤぬ 㸱㸬⬚⭢⭘
㸲㸬⓶Ⰽㄪኚ
㸳㸬ࡑࡢࡢ⮬ᕫᢠయ㝧ᛶ㸦ᢠࢭࢳࣝࢥࣜࣥཷᐜయᢠయࠊᢠ᰾ᢠయࠊᢠ⏥≧⭢ᢠయ࡞㸧
㹁㸬㚷ูデ᩿
Stiff-personೃ⩌ࠊ࣑࢜ࢺࢽೃ⩌ࠊMcAdle Definite㸸Aࡢࡍ࡚ࢆ‶ࡓࡋࠊCࢆ㚷ู࡛ࡁࡿࡶࡢ
Probable㸸A1ࡑࡢࡢA㡯┠2ࡘࢆ‶ࡓࡋࠊCࢆ㝖እ࡛ࡁࡿࡶࡢ Possible㸸A1Bࡢ1㡯┠௨ୖࢆ‶ࡓࡍࡶࡢ
㸦సᡂ㸸Ώ㑔ಟࡽ㸧
