WordNet

being long-lasting and recurrent or characterized by long suffering; "chronic indigestion"; "a chronic shortage of funds"; "a chronic invalid"
of long duration; "chronic money problems" (同)continuing
habitual; "a chronic smoker" (同)inveterate
characterized or caused by inflammation; "an inflammatory process"; "an inflammatory response"

PrepTutorEJDIC

(病気が)長期にわたる,慢性の / 《名詞の前にのみ用いて》常習の,癖になった
炎症を起こす,炎症性の / 憤激させる,扇動的な

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/01/12 19:06:41」(JST)

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated inflammatory disorder of the peripheral nervous system. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy (because it involves the nerve roots).^[1] CIDP is closely related to Guillain-Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. An asymmetrical variant of CIDP is known as Lewis-Sumner syndrome.^[2]

Overview

Chronic inflammatory demyelinating polyneuropathy^[3] is believed to be due to immune cells, which normally protect the body from foreign infection, incorrectly attacking the nerves in the body instead. As a result, the affected nerves fail to respond, or respond only weakly, to stimuli, causing numbing, tingling, pain, progressive muscle weakness, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations. The likelihood of progression of the disease is high.

CIDP is under-recognized and under-treated due to its heterogeneous presentation (both clinical and electrophysiological) and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery.^[4]

Lack of awareness and treatment of CIDP is also due to limitations of clinical trials. Although there are stringent research criteria for selecting patients to clinical trials, there are no generally agreed-on clinical diagnostic criteria for CIDP due to its different presentations in symptoms and objective data. Application of the present research criteria to routine clinical practice often miss the diagnosis in a majority of patients, and patients are often left untreated despite progression of their disease.^[5]

In some cases electrophysiological studies fail to show any evidence of demyelination. Though conventional electrophysiological diagnostic criteria are not met, the patient may still respond to immunomodulatory treatments. In such cases, presence of clinical characteristics suggestive of CIDP are critical, justifying full investigations, including sural nerve biopsy.^[6]

Diagnosis

Diagnosis is usually made through a clinical neurological examination. Patients usually present with a history of weakness, numbness, tingling, pain and difficulty in walking. They may additionally present with fainting spells while standing up or burning pain in extremities. Some patients may have sudden onset of back pain or neck pain radiating down the extremities, usually diagnosed as radicular pain. These symptoms are usually progressive and may be intermittent.

On examination the patients may have weakness, and loss of deep tendon reflexes (rarely increased or normal). There may be atrophy (shrinkage) of muscles, fasciculations (twitching) and loss of sensation. Patients may have Multi-Focal Motor neuropathy, as they have no sensory loss.

The patient may present with a single cranial nerve or peripheral nerve dysfunction.

Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems breathing, eye, bowel, bladder and cardiac problems.

Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.

Electrodiagnostics - electromyography (EMG) and nerve conduction study (NCS).

In usual CIDP, the nerve conduction studies show demyelination. These findings include:

a reduction in nerve conduction velocities;
the presence of conduction block or abnormal temporal dispersion in at least one motor nerve;
prolonged distal latencies in at least two nerves;
absent F waves or prolonged minimum F wave latencies in at least two motor nerves. (In some case EMG/NCV can be normal).

Serum test to exclude other autoimmune diseases.

Spinal tap and serum test for anti-ganglioside antibodies. These antibodies are present in the branch of CIDP diseases comprised by anti-GM1, anti-GD1a, and anti-GQ1b.

Sural nerve biopsy

Biopsy is considered for those patients in whom the diagnosis is not completely clear, when other causes of neuropathy (e.g., hereditary, vasculitic) cannot be excluded, or when profound axonal involvement is observed on EMG.

Treatment

First-line treatment for CIDP includes corticosteroids (e.g. prednisone), plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIG) which may be prescribed alone or in combination with an immunosuppressant drug.^[7]

IVIG and plasmapheresis have proven benefit in randomized, double-blind, placebo-controlled trials. Despite less definitive published evidence of efficacy, corticosteroids are considered standard therapies because of their long history of use and cost effectiveness. IVIG is probably the first-line CIDP treatment, but is extremely expensive (in the U.S., a single 65 g dose of Gamunex brand in 2010 might be billed at the rate of $8,000, just for the immunoglobulin, not including other charges such as nurse administration). Gamunex brand IVIG is the only U.S. FDA approved treatment for CIDP, as in 2008 Talecris, the maker of Gamunex, received orphan drug status for this drug for the treatment of CIDP.

Immunosuppressive drugs are often of the cytotoxic (chemotherapy) class, including rituximab (Rituxan) which targets B Cells, and cyclophosphamide, a drug which reduces the function of the immune system. Cyclosporin has also been used in CIDP but with less frequency as it is a newer approach.^[8] Cyclosporin is thought to bind to immunocompetent lymphocytes, especially T-lymphocytes.

Non-cytotoxic immunosuppressive treatments usually include the anti-rejection transplant drugs azathioprine (Imuran/Azoran) and mycophenolate mofetil (Cellcept). In the U.S., these drugs are used as "off-label" treatments for CIDP, meaning that their use here is accepted by the FDA, but that CIDP treatment is not explicitly indicated or approved in the drug literature. Before azathioprine is used, the patient should first have a blood test that ensures that azathioprine can safely be used.

Anti-thymocyte globulin (ATG), an immunosuppressive agent that selectively destroys T lymphocytes is being studied for use in CIDP. Anti-thymocyte globulin is the gamma globulin fraction of antiserum from animals that have been immunized against human thymocytes. It is a polyclonal antibody.

Although chemotherapeutic and immunosuppressive agents have shown to be effective in treating CIDP, significant evidence is lacking, mostly due to the heterogeneous nature of the disease in the patient population in addition to the lack of controlled trials.

Physical therapy may improve muscle strength, function and mobility, and minimize the shrinkage of muscles and tendons and distortions of the joints.

Prognosis

As in multiple sclerosis, another demyelinating condition, it is not possible to predict with certainty how CIDP is going to affect an individual in the future. The pattern of relapses and remissions varies greatly with each patient. A period of relapse can be very disturbing, but many patients make significant recoveries.

If diagnosed early, initiation of early treatment to prevent loss of nerve axons is recommended. However, many individuals are left with residual numbness, weakness, tremors, fatigue and other symptoms which can lead to long-term morbidity and diminished quality of life.^[1]

It is important to build a good relationship with doctors, both primary care and specialist. Because of the rarity of the illness, many doctors will not have encountered it before. Each case of CIDP is different, and relapses, if they occur, may bring new symptoms and problems. Because of the variability in severity and progression of the disease, doctors will not be able to give a definite prognosis. A period of experimentation with different treatment regimes is likely to be necessary in order to discover the most appropriate treatment regimen for a given patient.

References

^ ^a ^b Kissel JT (2003). "The treatment of chronic inflammatory demyelinating polyradiculoneuropathy". Semin Neurol 23 (2): 169–80. doi:10.1055/s-2003-41130. PMID 12894382.
^ http://www.cidpusa.org/LEWIS%20SUMMER.dwt
^ http://www.beverlyhillsneurology.com/cidp.html
^ Toothaker TB, Brannagan TH (2007). "Chronic inflammatory demyelinating polyneuropathies: current treatment strategies". Curr Neurol Neurosci Rep 7 (1): 63–70. doi:10.1007/s11910-007-0023-5. PMID 17217856.
^ Latov N (2002). "Diagnosis of CIDP". Neurology 59 (12 Suppl 6): S2–6. doi:10.1212/wnl.59.12_suppl_6.s2. PMID 12499464.
^ Azulay JP (2006). "[The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides]". Rev. Neurol. (Paris) (in French) 162 (12): 1292–5. PMID 17151528.
^ Hughes RA (2002). "Systematic reviews of treatment for inflammatory demyelinating neuropathy". Journal of Anatomy 200 (4): 331–9. doi:10.1046/j.1469-7580.2002.00041.x. PMC 1570692. PMID 12090400.
^ Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N (2005). "Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin". J. Neurol. Neurosurg. Psychiatr. 76 (8): 1115–20. doi:10.1136/jnnp.2003.035428. PMC 1739743. PMID 16024890.

External links

http://cidplog.com/ My CIDP Experience + Symptoms + Progression + Treatment
cidp at NINDS
CIDP information and booklets from the Guillain-Barré Syndrome Support Group (UK and Ireland)
The Neuropathy Association
Specialised reference centre for rare neuropathies
The Inflammatory Neuropathy Support Group of Victoria Inc.
GBS/CIDP Foundation International

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 慢性炎症性脱髄性多発神経炎：病因、臨床的特徴、および診断 chronic inflammatory demyelinating polyneuropathy etiology clinical features and diagnosis
2. 慢性炎症性脱髄性多発神経炎：治療および予後 chronic inflammatory demyelinating polyneuropathy treatment and prognosis
3. 免疫誘導性神経障害 immune mediated neuropathies
4. 小児における後天性末梢性ニューロパチーの概要 overview of acquired peripheral neuropathies in children
5. 成人におけるギラン・バレー症候群の臨床的特徴および診断 clinical features and diagnosis of guillain barre syndrome in adults

English Journal

Immunoadsorption with regenerating systems in neurological disorders - A single center experience.

Hohenstein B1, Passauer J2, Ziemssen T3, Julius U4.
Atherosclerosis. Supplements.Atheroscler Suppl.2015 May;18:119-23. doi: 10.1016/j.atherosclerosissup.2015.02.022.
In recent years, immunoadsorption is increasingly recognized as an alternative treatment approach replacing therapeutic plasma exchange in a variety of neurological disorders. While most experience is based on the application of single-use tryptophan adsorbers, less data exists on the application of
PMID 25936315

Elevation of plasma 1-deoxy-sphingolipids in type 2 diabetes mellitus: a susceptibility to neuropathy?

Dohrn MF1, Othman A, Hirshman SK, Bode H, Alecu I, Fähndrich E, Karges W, Weis J, Schulz JB, Hornemann T, Claeys KG.
European journal of neurology : the official journal of the European Federation of Neurological Societies.Eur J Neurol.2015 May;22(5):806-e55. doi: 10.1111/ene.12663. Epub 2015 Jan 26.
BACKGROUND AND PURPOSE: Diabetic distal sensorimotor polyneuropathy (DSPN) is a frequent, disabling complication of diabetes mellitus. There is increasing evidence that sphingolipids play a role in insulin resistance and type 2 diabetes (T2DM). Whether neurotoxic 1-deoxy-sphingolipids are elevated i
PMID 25623782

Impaired dendritic cell function in a spontaneous autoimmune polyneuropathy.

Quan S1, Kim HJ2, Dukala D1, Sheng JR1, Soliven B3.
Journal of immunology (Baltimore, Md. : 1950).J Immunol.2015 May 1;194(9):4175-84. doi: 10.4049/jimmunol.1401766. Epub 2015 Mar 30.
Spontaneous autoimmune polyneuropathy (SAP) in B7-2 knockout NOD mice mimics the progressive form of chronic inflammatory demyelinating polyradiculoneuropathy, and is mediated by myelin protein zero (P0)-reactive Th1 cells. In this study, we focused on the effect of B7-2 deletion on the function of
PMID 25825437

Japanese Journal

慢性炎症性脱髄性多発神経炎(CIDP) (特集整形外科疾患と鑑別を要する神経内科疾患)

三澤園子
整形・災害外科 57(13), 1709-1714, 2014-12
NAID 40020285829

難治性免疫性神経疾患における高額薬剤の使用について : 包括医療において負のインセンティブが働いているか?

荻野美恵子
北里医学 = Kitasato medicine 44(1), 7-12, 2014-06
NAID 40020140528

炎症性ニューロパチーの超音波診断 (特集神経筋疾患の超音波診断)

杉本太路,越智一秀,細見直永 [他]
Brain and nerve : 神経研究の進歩 66(3), 223-228, 2014-03
NAID 40020008757

Related Pictures

★リンクテーブル★

リンク元	「慢性炎症性脱髄性多発神経炎」「免疫介在性ニューロパチー」「慢性炎症性脱髄性多発神経障害」
関連記事	「inflammatory」「demyelinating polyneuropathy」「chronic」「demyelinating」

「慢性炎症性脱髄性多発神経炎」

Structure of a typical neuron
Neuron
	Dendrite Soma Axon Nucleus Node of; Ranvier Axon terminal Schwann cell Myelin sheath

Chronic inflammatory demyelinating polyneuropathy
Classification and external resources
ICD-9	357.81
OMIM	139393
DiseasesDB	30084
eMedicine	neuro/467
MeSH	D020277

　　[★]

英: chronic inflammatory demyelinating polyradiculoneuropathy, chronic inflammatory demyelinating polyneuropathy, CIDP
同: 慢性炎症性脱髄性多発根神経炎、慢性炎症性脱髄性多発ニューロパチー、慢性炎症性脱髄性多発性根神経障害、慢性炎症性脱髄性多発神経障害、慢性炎症性脱髄性多発根神経炎
関: 難病

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慢性炎症性脱髄性多発ニューロパシー : 48 件
慢性炎症性脱髄性多発ニューロパチー : 約 131,000 件
慢性炎症性脱髄性多発根ニューロパシー : 約 13,500 件
慢性炎症性脱髄性多発根ニューロパチー : 約 13,500 件
慢性炎症性脱髄性多発神経炎 : 約 19,200 件
慢性炎症性脱髄性多発性神経炎 : 77 件
慢性炎症性脱髄性多発神経根炎 : 75 件
慢性炎症性脱髄性多発性神経根炎 : 3 件
慢性炎症性脱髄性多発根神経炎 : 約 84,100 件
慢性炎症性脱髄性多発性根神経炎 : 31 件
慢性炎症性脱髄性多発神経障害 : 42 件
慢性炎症性脱髄性多発性神経障害 : 12 件
慢性炎症性脱髄性多発根神経障害 : 17 件
慢性炎症性脱髄性多発性根神経障害 : 13 件
慢性炎症性脱髄性多発神経根障害 : 8 件
慢性炎症性脱髄性多発性神経根障害 : nothing

概念

難病であり、特定疾患治療研究事業に指定されている疾患である。
左右対称性の四肢の遠位・近位筋の筋力低下・感覚障害を慢性(2ヶ月以上)の経過できたす原因不明の末梢神経疾患。
急性炎症性脱髄性多発根神経炎(AIDP)(ギラン・バレー症候群など)と対比される疾患である。

病因

末梢神経ミエリンの構成成分に対する免疫異常に基づく自己免疫性疾患と考えられている。

uptodateの見解

参考2

CIDPが単一の疾患なのか症候群なのかは議論が分かれるところである。以下の疾患はいずれも慢性、脱髄、炎症、あるいは免疫の媒介(immune-mediation)を共通に有する　(略)　(以下、鑑別疾患に上げられると思われるため、列挙。現時点でのCIDPの疾患概念はとにかく原因不明ということ)

CIDP
多巣性運動ニューロパチー、multifocal motor neuropathy、MMN
Lewis-Sumner症候群、Lewis-Sumner syndrome、MADSAM
IgM異常蛋白をともなう遠位脱髄性神経炎±抗MAG抗体、distal demyelinating neuropathy with IgM paraprotein, with or without anti-myelin associated glycoprotein
IgG, IgA異常蛋白をともなう脱髄性神経炎、demyelinating neuropathy with IgG or IgA paraprotein
POEMS症候群、POEMS syndrome
感覚優位型脱髄性神経炎、sensory predominant demyelinating neuropathy
中枢神経系の脱髄を伴う脱髄性神経炎、demyelinating neuropathy with central nervous system demyelination
その他の膠原病・血管炎、甲状腺中毒症、臓器移植・骨髄移植、遺伝性ニューロパチー、ネフローゼ症候群、炎症性腸疾患。
全身疾患に関連する脱髄性神経炎：B型肝炎、C型肝炎、HIV感染、リンパ腫、糖尿病、全身性エリテマトーデス

検査

脳脊髄検査：蛋白細胞解離

診断基準

参考1

診断：( 1.の1)2) ) and ( 2.の1) ) and ( 2.の2)~5)のうち１つ )

主要項目

1. 発症と経過

1) 2ヶ月以上の経過の、寛解・増悪を繰り返すか、慢性進行性の経過をとる多発ニューロパチーである。
2) 当該患者の多発ニューロパチーを説明できる明らかな基礎疾患、薬物使用、毒物への曝露がなく、類似疾患の遺伝歴がない。

2. 検査所見

1) 末梢神経伝導検査で、2本以上の運動神経において、脱髄を示唆する所見を示す。(伝導速度の低下、伝導ブロックまたは時間的分散の存在、遠位潜時の延長、F波欠如または最短潜時の延長)
2) 脳脊髄液検査で、蛋白増加をみとめ、細胞数は10/mm3未満である。
3) 免疫グロブリン大量療法、副腎皮質ステロイド薬、血液浄化療法、その他の免疫療法などにより改善を示した病歴がある。
4) MRIで神経根あるいは馬尾の肥厚または造影所見がある。
5) 末梢神経生検で脱髄を示唆する所見がある。

鑑別診断

1. 全身性疾患等による末梢神経障害：糖尿病、アミロイドーシス、膠原病、血管炎、悪性腫瘍、多発性骨髄腫、中枢神経系脱髄疾患、HIV感染症、サルコイドーシス
2) 末梢神経障害を起こす薬物への曝露
3) 末梢神経障害を起こす毒物への曝露
4) 末梢神経障害を起こす遺伝性疾患

治療

副腎皮質ステロイド、免疫グロブリン大量投与

参考

1. 慢性炎症性脱髄性多発神経炎（公費対象） - 難病情報センター

http://www.nanbyou.or.jp/entry/333

2. [charged] Chronic inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis - uptodate [1]
3. [charged] Chronic inflammatory demyelinating polyneuropathy: Treatment and prognosisFind Print Email - uptodate [2]