BRAIN and NERVE－神経研究の進歩74巻5号

2022年05月発行

増大特集 次の一手—神経筋疾患難治例をどのように治療するか

3種類のmainstay治療に反応しないCIDP—鑑別ポイントと治療戦略

著者： 飯島正博¹

所属機関： ¹名古屋大学医学部附属病院先端医療開発部

ページ範囲：P.517 - P.524

文献概要

慢性炎症性脱髄性多発根ニューロパチー（CIDP）は多様な病態を包含する症候群であり，治療反応性の違いはそれを強く裏付ける。Mainstay治療に抵抗性を示す多くはDADSやMADSAMをはじめとするatypical CIDPであり，特にIgG4自己抗体を病因とする一群は難治性CIDPとして知られる。既存治療では単純血漿交換療法と副腎皮質ステロイドの併用が一定の効果を示すが，将来的にはリツキシマブによる治療開発が期待される。経静脈的免疫グロブリン（IVIg）維持療法下でも短期間で再発を繰り返す免疫グロブリン製剤への治療依存例では，ステロイドや免疫抑制薬のadd-onの効果が期待できる。またこうした症例ではIVIgから皮下注用免疫グロブリン製剤（SCIg）による維持療法のオプション変更も有効である。IgG4サブクラス自己抗体は病因であり病勢を判断し得るバイオマーカーの側面を有する。またNfLは治療適正期を判断するとともに治療効果を判定するうえで有効性が期待できるバイオマーカーである。

参考文献

1）Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, et al: Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 50: 621-637, 1975

2）Gorson KC, Allam G, Ropper AH: Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 48: 321-328, 1997

3）Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, et al: European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force-Second revision. Eur J Neurol 28: 3556-3583, 2021

4）Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, et al: Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry 90: 125-132, 2019

5）Sommer C, Koch S, Lammens M, Gabreels-Festen A, Stoll G, et al: Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP. Neurology 65: 1924-1929, 2005

6）Hughes RA, Mehndiratta MM, Rajabally YA: Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 11: CD002062, 2017［doi: 10.1002/14651858.CD002062.pub4］

7）Oaklander AL, Lunn MP, Hughes RAC, van Schaik IN, Frost C, et al: Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev 1: CD010369, 2017［doi: 10.1002/14651858.CD010369.pub2］

8）Mehndiratta MM, Hughes RAC, Pritchard J: Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 1: CD003906, 2015［doi: 10.1002/14651858.CD010369.pub2］

9）Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN: Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev: CD001797, 2013［doi: 10.1002/14651858.CD001797.pub2］

10）Doneddu PE, Dentoni M, Nobile-Orazio E: Atypical chronic inflammatory demyelinating polyradiculoneuropathy: recent advances on classification, diagnosis, and pathogenesis. Curr Opin Neurol 34: 613-624, 2021

11）Van den Bergh PYK, Hadden RDM, Bouche P, Cornblath DR, Hahn A, et al: European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society, first revision. Eur J Neurol 17: 356-363, 2010

12）Eftimov F, Vermeulen M, van Doorn PA, Brusse E, van Schaik IN: Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology 78: 1079-1084, 2012

13）小池春樹, 祖父江 元: 家族性アミロイドポリニューロパチー—臨床と病理. Brain Nerve 66: 749-762, 2014

14）Tomita M, Koike H, Kawagashira Y, Iijima M, Adachi H, et al: Clinicopathological features of neuropathy associated with lymphoma. Brain 136: 2563-2578, 2013

15）Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ: Distal acquired demyelinating symmetric neuropathy. Neurology 54: 615-620, 2000

16）Saperstein DS, Amato AA, Wolfe GI, Katz JS, Nations SP, et al: Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve 22: 560-566, 1999

17）Joint Task Force of the EFNS and the PNS: European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. J Peripher Nerv Syst 10: 220-228, 2005

18）Querol L, Nogales-Gadea G, Rojas-Garcia R, Diaz-Manera J, Pardo J, et al: Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology 82: 879-886, 2014

19）Kadoya M, Kaida K, Koike H, Takazaki H, Ogata H, et al: IgG4 anti-neurofascin155 antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: clinical significance and diagnostic utility of a conventional assay. J Neuroimmunol 301: 16-22, 2016

20）Menon D, Katzberg HD, Bril V: Treatment approaches for atypical CIDP. Front Neurol 12: 653734, 2021［doi: 10.3389/fneur.2021.653734］

21）van Schaik IN, Eftimov F, van Doorn PA, Brusse E, van den Berg LH, et al: Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol 9: 245-253, 2010

22）Nobile-Orazio E, Cocito D, Jann S, Uncini A, Beghi E, et al: Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol 11: 493-502, 2012

23）Querol L, Rojas-García R, Diaz-Manera J, Barcena J, Pardo J, et al: Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm 2: e149, 2015［doi: 10.1212/NXI.0000000000000149］

24）Shimizu S, Iijima M, Fukami Y, Tamura N, Nakatochi M, et al: Efficacy and safety of rituximab in refractory CIDP with or without IgG4 autoantibodies (RECIPE): protocol for a double-blind, randomized, placebo-controlled clinical trial. JMIR Res Protoc 9: e17117, 2020［doi: 10.2196/17117］

25）Adrichem ME, Bus SR, Wieske L, Mohammed H, Verhamme C, et al: Combined intravenous immunoglobulin and methylprednisolone as induction treatment in chronic inflammatory demyelinating polyneuropathy (OPTIC protocol): a prospective pilot study. Eur J Neurol 27: 506-513, 2020

26）Dalakas MC, Spaeth PJ: The importance of FcRn in neuro-immunotherapies: from IgG catabolism, FCGRT gene polymorphisms, IVIg dosing and efficiency to specific FcRn inhibitors. Ther Adv Neurol Disord 14: 1756286421997381, 2021［doi: 10.1177/1756286421997381］

27）Fukami Y, Iijima M, Koike H, Yamada S, Hashizume A, et al: Association of serum neurofilament light chain levels with clinicopathology of chronic inflammatory demyelinating polyneuropathy, including NF155 reactive patients. J Neurol 268: 3835-3844, 2021