WordNet

myositis characterized by weakness of limb and neck muscles and much muscle pain and swelling; progression and severity vary among individuals

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/02/27 23:39:32」(JST)

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Polymyositis (PM)("inflammation of many muscles") is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis.

Epidemiology

Polymyositis, like dermatomyositis, strikes females with greater frequency than males.

Signs and symptoms

The hallmark of polymyositis is weakness and/or loss of muscle mass in the proximal musculature, as well as flexion of the neck and torso.^[1] These symptoms can be associated with marked pain in these areas as well. The hip extensors are often severely affected, leading to particular difficulty in ascending stairs and rising from a seated position. The skin involvement of dermatomyositis is absent in polymyositis. Dysphagia (difficulty swallowing) or other problems with esophageal motility occur in as many as 1/3 of patients. Low grade fever and peripheral adenopathy may be present. Foot drop in one or both feet can be a symptom of advanced polymyositis and inclusion body myositis. The systemic involvement of polymyositis includes interstitial lung disease and cardiac disease, such as heart failure and conduction abnormalities.^[2]

Polymyositis tends to become evident in adulthood, presenting with bilateral proximal muscle weakness often noted in the upper legs due to early fatigue while walking. Sometimes the weakness presents itself as an inability to rise from a seated position without help or an inability to raise one's arms above one's head. The weakness is generally progressive, accompanied by lymphocytic inflammation (mainly cytotoxic T cells).

Associated Illnesses

Polymyositis and the associated inflammatory myopathies have an associated increased risk of malignancy.^[3] The features they found associated with an increased risk of cancer was older age, age greater than 45, male sex, dysphagia, cutaneous necrosis, cutaneous vasculitis, rapid onset of myositis (<4 weeks), elevated CK, higher ESR, higher CRP levels. Several factors were associated with lower-than-average risk, including the presence of ILD, arthritis/arthralgia, Raynaud's syndrome, or anti-Jo-1 antibody.^[3] The malignancies that are associated are nasopharyngeal cancer, lung cancer, non-hodgkins lymphoma & bladder cancer amongst others.^[4]

Cardiac involvement manifests itself typically as heart failure, and is present in up to 77% of patients.^[2] Interstitial lung disease is found in up to 65% of patients with polymyositis, as defined by HRCT or restrictive ventilatory defects compatible with ILD.^[5]

Causes

Polymyositis is an inflammatory myopathy mediated by cytotoxic T cells with an as yet unknown autoantigen, while dermatomyositis is a humorally mediated angiopathy resulting in myositis and a typical dermatitis.^[6] The cause of polymyositis is unknown and may involve viruses and autoimmune factors. Cancer may trigger polymyositis and dermatomyositis, possibly through an immune reaction against cancer that also attacks a component of muscles.^[7]

Diagnosis

Diagnosis is fourfold, history and physical examination, elevation of creatine kinase, electromyograph (EMG) alteration, and a positive muscle biopsy.^[8]

The hallmark clinical features of polymyositis is proximal muscle weakness, with less important findings being muscle pain and dysphagia. Cardiac and pulmonary findings will be present in approximately 25% of cases of patients with polymyositis.

Sporadic inclusion body myositis (sIBM): IBM is often confused with (misdiagnosed as) polymyositis or dermatomyositis that does not respond to treatment is likely IBM. sIBM comes on over months to years; polymyositis comes on over weeks to months. Polymyositis tends to respond well to treatment, at least initially; IBM does not.

Treatment

The first line treatment for polymyositis is corticosteroids. Specialized exercise therapy may supplement treatment to enhance quality of life.

Notable cases

Dan Christensen, painter of abstract art. Died due to heart failure caused by polymyositis.^[9]
Robert Erickson, American composer and teacher who was a leading modernist exponent of "12-tone" composition. Died from the effects of polymyositis.^[10]
David Lean, film director.^[11]^[12]

References

^ Kenneth W. Strauss, Hermann Gonzalez-Buritica, Munther A. Khamashta, Graham R.V. Hughes (1989). "Polymyositis-dermatomyositis: a clinical review". Postgraduate Medical Journal 65: 437–443. doi:10.1136/pgmj.65.765.437.
^ ^a ^b Zhang Lu, Wang Guo-chun, Ma Li, Zu Ning (2012). "Cardiac Involvement in Adult Polymyositis or Dermatomyositis: A Systematic Review". Clinical Cardiology 35 (11): 686–691. doi:10.1002/clc.22026.
^ ^a ^b Xin Lu, Hanbo Yang, Xiaoming Shu, Fang Chen, Yinli Zhang, Sigong Zhang, Qinglin Peng, Xiaolan Tian, Guochun Wang (2014). "Factors Predicting Malignancy in Patients with Polymyositis and Dermatomyostis: A Systematic Review and Meta-Analysis". PLOS ONE 9 (4): e94128. doi:10.1371/journal.pone.0094128.
^ Hill, C.L.; Zhang, Y.; Sigurgeirsson, B.; Pukkala, E.; Mellemkjaer, L.; Airio, A.; Evans, S.R.; Felson, D.T. (January 2001). "Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study". Lancet 357 (9250): 96–100. doi:10.1016/S0140-6736(00)03540-6. PMID 11197446. Retrieved 20 April 2015.
^ M Fathi, M Dastmalchi, E Rasmussen, I E Lundberg, G Tornling (2004). "Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis". Ann Rheum Dis 63: 297–301. doi:10.1136/ard.2003.006122.
^ Gerald J D Hengstman, Baziel G M van Engelen (2004). "Polymyositis, invasion of non-necrotic muscle fibres, and the art of repetition". British Medical Journal 329: 1464–1467. doi:10.1136/bmj.329.7480.1464.
^ Hajj-ali, Rula A. (August 2013). "Polymyositis and Dermatomyositis". Merck Manual Home Edition. Retrieved 20 April 2015.
^ SCOLA, ROSANA HERMINIA, WERNECK, LINEU CESAR, PREVEDELLO, DANIEL MONTE SERRAT, TODERKE, EDIMAR LEANDRO, & IWAMOTO, FÁBIO MASSAITI. (2000). "Diagnosis of dermatomyositis and polymyositis: a study of 102 cases.". Arquivos de Neuro-Psiquiatria 58: 789–799. doi:10.1590/S0004-282X2000000500001.
^ "Dan Christensen, 64, Painter of Abstract Art, Dies". The New York Times. 27 January 2007. Retrieved 20 April 2015.
^ "Obutiary - Robert Erickson". SF Gate. 29 April 1997. Retrieved 20 April 2015.
^ Stevens, Jr., George (2006). Conversations with the Great Moviemakers of Hollywood's Golden Age at the American Film Institute. Knopf. p. 427. ISBN 978-1-4000-4054-4.
^ Brownlow, Kevin (1996). David Lean: A Biography. Macmillan. pp. 1466–1467. ISBN 978-1-4668-3237-4. Retrieved 20 April 2015.

External links

The Myositis Association

UpToDate Contents

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1. 成人における皮膚筋炎および多発性筋炎の診断および鑑別診断 diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults
2. 成人における皮膚筋炎および多発性筋炎の臨床症状 clinical manifestations of dermatomyositis and polymyositis in adults
3. 成人における皮膚筋炎および多発性筋炎の初期治療 initial treatment of dermatomyositis and polymyositis in adults
4. 成人における再発および抵抗性皮膚筋炎と多発性筋炎の治療 treatment of recurrent and resistant dermatomyositis and polymyositis in adults
5. 若年性皮膚筋炎および多発性筋炎の病因および臨床症状 pathogenesis and clinical manifestations of juvenile dermatomyositis and polymyositis

English Journal

Nailfold capillaroscopic changes in dermatomyositis and polymyositis.

Manfredi A1, Sebastiani M, Cassone G, Pipitone N, Giuggioli D, Colaci M, Salvarani C, Ferri C.
Clinical rheumatology.Clin Rheumatol.2015 Feb;34(2):279-84. doi: 10.1007/s10067-014-2795-8. Epub 2014 Oct 17.
Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study wa
PMID 25318613

Polymyositis/dermatomyositis and Malignancy Risk: A Metaanalysis Study.

Yang Z1, Lin F1, Qin B1, Liang Y2, Zhong R1.
The Journal of rheumatology.J Rheumatol.2015 Feb;42(2):282-91. doi: 10.3899/jrheum.140566. Epub 2014 Dec 1.
OBJECTIVE: To investigate the association between polymyositis (PM)/dermatomyositis (DM) and risks of malignancy.METHODS: We searched Pubmed for articles dated before August 16, 2013. Studies were included if they met the following criteria: (1) a cohort or observational study; (2) PM or DM as one o
PMID 25448790

Echocardiographic Abnormalities in New-onset Polymyositis/dermatomyositis.

Péter A1, Balogh Á2, Szilágyi S2, Faludi R2, Nagy-Vincze M2, Édes I2, Dankó K2.
The Journal of rheumatology.J Rheumatol.2015 Feb;42(2):272-81. doi: 10.3899/jrheum.140626. Epub 2014 Nov 29.
OBJECTIVE: To identify early echocardiographic abnormalities at the time of diagnosis of polymyositis (PM) and dermatomyositis (DM) and follow the echocardiographic findings during the first 3 months of therapy.METHODS: We included 30 PM/DM patients (23/7) with a mean age of 42.3 ± 1.6 years and wi
PMID 25433528

Japanese Journal

自己免疫性筋炎における骨格筋ミオシン架橋C蛋白の免疫原性 (特集自己免疫疾患の発症誘導要因)

沖山奈緒子
臨床免疫・アレルギー科 58(1), 53-56, 2012-07-00
NAID 40019390304

重症多発筋炎を合併した精巣腫瘍の1例

亀井潤,京野陽子,山田幸央 [他],篠原充,本間之夫
泌尿器科紀要 58(7), 361-364, 2012-07-00
… At the same time, creatine kinase was extremely elevated and he was diagnosed with polymyositis (PM). … After the third chemotherapy session, the testicular tumor was in complete remission and he had no symptoms of polymyositis. …
NAID 40019362594

Effects of intravenous immunoglobulin therapy in Japanese patients with polymyositis and dermatomyositis resistant to corticosteroids : a randomized double-blind placebo-controlled trial

MIYASAKA Nobuyuki,HARA Masako,KOIKE Takao,SAITO Eizo,YAMADA Masahito,TANAKA Yoshiya,Additional Members of the GB-0998 Study Group
Modern rheumatology 22(3), 382-393, 2012-06-01
NAID 10030782212

Related Pictures

Polymyositis - Causes, Symptoms, Prognosis, Diagnosis, Treatment Medical Videos ppt and Lecture Notes: Dermatology Polymyositis - YouTube Polymyositis | Myositis Support and Understanding .what is polymyositis | Polymyositis | Pinterest Polymyositis - Wikipedia Polymyositis / Dermatomyositis - MSK - Medbullets Step 1

★リンクテーブル★

リンク元	「100Cases 14」「多発性筋炎、皮膚筋炎」「多発性筋炎」「PM」
拡張検索	「polymyositis-dermatomyositis」「idiopathic polymyositis」「interstitial nodular polymyositis」

「100Cases 14」

Polymyositis
	Micrograph of polymyositis. Muscle biopsy. H&E stain.
Classification and external resources
Specialty	Rheumatology
ICD-10	M33.2
ICD-9-CM	710.4
DiseasesDB	10343
MedlinePlus	000428
eMedicine	med/3441 emerg/474
MeSH	D017285
	[edit on Wikidata]

　　[★]

☆case14　複視

■glossary

diplopia n. 複視

筋力低下、筋無力、筋脱力 muscle weakness, muscular weakness

sunken

vt. sinkのpp.

adj.

沈没した、沈んだ、水中の

沈下した、一段低いところにある

落ち込んだ、くぼんだ

3,4-ジアミノピリジン 3,4-diaminopyridine 3,4-DAP K+チャネル阻害薬;ランバート・イートン筋無力症治療

■症例

43-year-old woman

cheif complaint: diplopia

present history: diplopia and holding her head up ; more marked in the evenings, for the last 3 months. difficulty of chewing. voice has become quieter. weight loss (3kg / 6months). non-smoker. drinks about 15 units/week. no regular medication.

past history: no significant previous medical illnesses.

family history: lives with her husband and three children.

・診察 examination

looks well.

organ systems: normal; cardiovascular, respiratory, and abdominal systems.

muscle power; grossly normal. decrease after testing a movement repetitively.

motor function: normal; tone, coordination, reflexes and sensation.

bilateral ptosis. exacerbated by prolonged upward gaze

eye: normal; pupillary reflexes, eye movements, and funduscopy

■答え

diagnosis: myathenia gravis

differential diagnoses:

CASES

上位and/or下位運動ニューロン motor neurone disease　運動ニューロン疾患：線維束性攣縮。進行例では筋力低下

筋 muscular dystrophy　筋ジストロフィー：ある種の筋肉が選択的に筋力低下する。家族歴がある。

筋 dystrophia myotonica　強直性筋ジストロフィー：咬筋、側頭筋、胸鎖乳突筋の筋萎縮、四肢遠位端の筋萎縮。顔貌が特徴的(前頭部脱毛、無表情、窪んだ頬)。家族歴ある。筋電図が診断に有用(急降下爆撃音)。

筋 polymyositis　多発筋炎：普通は皮疹と関節痛が出現。CKが上昇。筋生検が診断に有用

筋 myopathy　ミオパチー：甲状腺中毒性ミオパチー、甲状腺機能低下症によるミオパチー、クッシング症候群によるミオパチー、アルコール性のミオパチー

神経筋接合部？ non-metastatic associations of malignancy (paraneoplastic syndrome(傍腫瘍性症候群 = 腫瘍随伴症候群)のこと)：胸腺腫の症例の10%に重症筋無力症がみられる。ランバート・イートン筋無力症症候群は小細胞癌と関連がある。

HIM.2674

神経筋接合部 congenital myasthenia syndrome　先天性筋無力症症候群
神経筋接合部 drug-induced myasthenia　薬剤性筋無力症

Treatment with penicillamine (used for scleroderma or rheumatoid arthritis) may result in true autoimmune MG, but the weakness is usually mild, and recovery occurs within weeks or months after discontinuing its use.

　重症筋無力症の誘発：ペニシラミン(強皮症や関節リウマチの治療に用いられる)。

　　mildだし、薬剤の中断で改善する。

Aminoglycoside antibiotics or procainamide can cause exacerbation of weakness in myasthenic patients; very large doses can cause neuromuscular weakness in normal individuals.

　重症筋無力症の悪化：アミドグリコシド系抗菌薬、プロカインアミド

　　MGの患者の筋脱力が悪化する。

神経筋接合部 Lambert-Eaton myasthenic syndrome　ランバート・イートン筋無力症症候群：全身の筋肉が冒されるが、特に下肢の近位筋が冒される。MGと同じように～70%の患者で脳神経所見(眼瞼下垂、複視など)が認められる。MGと違うのは(1)反射が消失・減弱すること、(2)自律神経系の変化(口渇、勃起不全)を生じる、(3)神経刺激検査で漸増(waxing)が見られることである。病因は神経筋接合部のP/Q type calcium channelsに対する抗体の出現であり、85%の患者で見いだされる。治療はMGのように血漿交換や免疫抑制薬が使われる。3,4-DAPやpyridostigmineは症状に対する治療のために用いる。前者は運動神経の終末部でカルシウムチャネルをブロックし活動電位を延長させる。後者はアセチルコリンエステラーゼを阻害してシナプスにおける神経伝達物質の濃度を上げる。
精神疾患 neurasthenia　神経衰弱症：歴史的な用語。器質的な障害を伴わない筋無力症のような脱力を伴う症候群。患者は筋脱力や疲労を訴えてやってくる。筋肉の検査では器質的変化は認めないが"jerky release"あるいは"give-away weakness"が認められる。患者の主訴は反復動作による筋力低下よりむしろ疲労や感情鈍麻である。
内分泌疾患 hyperthyroidism　甲状腺機能亢進症：MGが疑われる患者にはthyroid function testをルーチンにやる。甲状腺機能異常は筋無力症の筋力低下を大きくすることがある。
神経筋接合部 botulism　ボツリヌス症：ボツリヌス毒素はシナプス前膜からの神経伝達物質の開口分泌を妨げる。症状はbulbar weakness (複視、構音障害、嚥下困難)。感覚障害はない。深部腱反射は初期には保たれている。進行すれば反射は見られなくなる。筋脱力は全身性。呼吸困難に陥ることがある。精神状態は正常。自律神経症状(麻痺性イレウス、便秘、urinary retention、瞳孔の散大、瞳孔の反応性低下、口渇)。確定診断は血清中の毒素の検出だけど、見つかることはまれ。神経伝導検査(nerve conduction studies)：compound muscle action potentials (CMAPs)の低下。高頻度の刺激で振幅が増加。治療：intubation for airway protection、呼吸補助、aggressive inpatient supportive care(e.g., nutrition, DVT prophylaxis)。馬の抗毒素を検査結果が帰ってくる前に投与する(？)。
上位運動ニューロン intracranial mass lesions　頭蓋内占拠病変：複視はintracranial mass lesionが外眼筋の神経を圧迫することにより生じる。
筋 progressive external ophthalmoplegia　進行性外眼筋麻痺：外眼筋の筋脱力を伴う。四肢の近位筋の筋力低下やそのほかの全身症状を伴うことがある。ミトコンドリアの異常を有する。

■参考文献

HIM = Harrison's Principles of Internal Medicine 17th Edition

CASES = 100 Cases in Clinical Medicine Second edition

IMD = 内科診断学第2版

「多発性筋炎、皮膚筋炎」

　　[★]

多発性筋炎 polymyositis PM; 皮膚筋炎 dermatomyositis DM
関: 膠原病

概念

疫学

症状

初発症状は皮疹、発熱、全身倦怠感、筋力低下などが多い。

病態	レイノー現象	抗核抗体	リウマトイド因子	抗好中球細胞質抗体	皮疹	皮下結節	関節炎	筋炎	漿膜炎	自己抗体
病態										Jo-1

病理	壊死性血管炎	糸球体腎炎	間質性肺炎	心炎	唾液腺炎	オニオンスキン病変	ワイヤーループ病変	ヘマトキシリン体	LE細胞
病理

筋症状(amyopathic dermatomyositisを除く)

四肢近位筋群、頚部筋、咽頭部(嚥下障害、発声障害につながる)などの対称性筋力低下。筋痛。筋萎縮。

皮膚症状(多発性筋炎を除く)

ヘリオトロープ皮疹：特異皮疹
ゴットロン徴候：特異皮疹：
ポイキロデルマ：体幹、頚部などにみられるそう痒性紅斑で経過とともに萎縮、網状色素沈着および脱失、毛細血管拡張をみる
時に、顔面蝶形紅斑や爪上皮内出血、レイノー症状
皮下石灰沈着

臓器障害

時に間質性肺炎、肺線維症、
心筋炎

検査

血清筋原性酵素(CK)：増加
筋電図：筋病変を示唆
自己抗体検査

抗Jo-1抗体：感度低いが、特異度は高い　PMの方がDMより陽性率が高い
抗核抗体：40-50%の症例でみられる(IMD)

診断

鑑別診断

参考2

感染による筋炎、薬剤誘発性ミオパチー、内分泌異常に基づくミオパチー、筋ジストロフィーその他の先天性筋疾患

合併症

多発性筋炎/皮膚筋炎の発症前後に悪性腫瘍を合併しうる

参考

1. 多発性筋炎・皮膚筋炎 - 難病情報センター

http://www.nanbyou.or.jp/entry/293

2. 認定基準

http://www.nanbyou.or.jp/upload_files/067z_s.pdf

国試

「多発性筋炎」

　　[★]

英: polymyositis, PM
同: (国試)多発筋炎
関: 皮膚筋炎

[show details]

多発筋炎 : 約 18,700 件
多発性筋炎 : 約 272,000 件

→多発性筋炎、皮膚筋炎

症候

近位筋の筋力低下・萎縮、筋痛、筋把握痛
四肢の筋力低下→Gowers徴候

診断基準

PMはBohanとPeterの基準により以下の項目を満たすかどうかで診断する。

1) 対称性近位筋力低下
2) 筋生検による筋炎の存在
3) 血中筋逸脱酵素の上昇
4) 筋原性の筋電図変化

1〜4の全てを満たす場合define、3項目を満たす場合probable、2項目を満たす場合possible

「PM」

　　[★]

「polymyositis-dermatomyositis」

　　[★]

多発筋炎・皮膚筋炎

関: dermatomyositis

「idiopathic polymyositis」

　　[★]

特発性多発性筋炎

関: polymyositis

「interstitial nodular polymyositis」

　　[★] 間質結節性多発性筋炎

[strauss1989-1] Kenneth W. Strauss, Hermann Gonzalez-Buritica, Munther A. Khamashta, Graham R.V. Hughes (1989). "Polymyositis-dermatomyositis: a clinical review". Postgraduate Medical Journal 65: 437–443. doi:10.1136/pgmj.65.765.437.

[LU2012-2] Zhang Lu, Wang Guo-chun, Ma Li, Zu Ning (2012). "Cardiac Involvement in Adult Polymyositis or Dermatomyositis: A Systematic Review". Clinical Cardiology 35 (11): 686–691. doi:10.1002/clc.22026.

[LU2014-3] Xin Lu, Hanbo Yang, Xiaoming Shu, Fang Chen, Yinli Zhang, Sigong Zhang, Qinglin Peng, Xiaolan Tian, Guochun Wang (2014). "Factors Predicting Malignancy in Patients with Polymyositis and Dermatomyostis: A Systematic Review and Meta-Analysis". PLOS ONE 9 (4): e94128. doi:10.1371/journal.pone.0094128.

[4] Hill, C.L.; Zhang, Y.; Sigurgeirsson, B.; Pukkala, E.; Mellemkjaer, L.; Airio, A.; Evans, S.R.; Felson, D.T. (January 2001). "Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study". Lancet 357 (9250): 96–100. doi:10.1016/S0140-6736(00)03540-6. PMID 11197446. Retrieved 20 April 2015.

[FATHI2004-5] M Fathi, M Dastmalchi, E Rasmussen, I E Lundberg, G Tornling (2004). "Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis". Ann Rheum Dis 63: 297–301. doi:10.1136/ard.2003.006122.

[HENGSTMAN2004-6] Gerald J D Hengstman, Baziel G M van Engelen (2004). "Polymyositis, invasion of non-necrotic muscle fibres, and the art of repetition". British Medical Journal 329: 1464–1467. doi:10.1136/bmj.329.7480.1464.

[7] Hajj-ali, Rula A. (August 2013). "Polymyositis and Dermatomyositis". Merck Manual Home Edition. Retrieved 20 April 2015.

[SCOLA2000-8] SCOLA, ROSANA HERMINIA, WERNECK, LINEU CESAR, PREVEDELLO, DANIEL MONTE SERRAT, TODERKE, EDIMAR LEANDRO, & IWAMOTO, FÁBIO MASSAITI. (2000). "Diagnosis of dermatomyositis and polymyositis: a study of 102 cases.". Arquivos de Neuro-Psiquiatria 58: 789–799. doi:10.1590/S0004-282X2000000500001.

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polymyositis