WordNet

extremely precise with great attention to details; "examined it with microscopic care"
of or relating to or used in microscopy; "microscopic analysis"; "microscopical examination" (同)microscopical
so small as to be invisible without a microscope; "differences were microscopic" (同)microscopical
visible under a microscope; using a microscope (同)microscopical
inflammation of several arteries

PrepTutorEJDIC

顕微鏡の,顕微鏡による / 《しばしば《話》》非常に小さい,微細な / 非常に綿密な

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/03/11 16:22:19」(JST)

wiki en

Microscopic polyangiitis (also known as "Microscopic polyarteritis,"^[1] "Microscopic polyarteritis nodosa,"^[1] "MPA"^[2]) is an ill-defined autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.

§Signs and symptoms

Clinical features may include constitutional symptoms like fever, loss of appetite, weight loss, fatigue, and kidney failure.^[3] A majority of patients may have blood in the urine and protein in the urine. Rapidly progressive glomerulonephritis may occur. Because many different organ systems may be involved, a wide range of symptoms are possible in MPA.

Purpura and livedo may be present.^[4]

§Diagnosis

Laboratory tests may reveal an increased sedimentation rate, elevated CRP, anemia and elevated creatinine due to renal impairment. An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity^[5] (a constituent of neutrophil granules), and protein and red blood cells in the urine.

In patients with neuropathy, electromyography may reveal a sensorimotor peripheral neuropathy.

§Cause

While the mechanism of disease has yet to be fully elucidated, the leading hypothesis is that the process is begun with an autoimmune process of unknown etiology that triggers production of p-ANCA. These antibodies will circulate at low levels until a pro-inflammatory trigger — such as infection, malignancy, or drug therapy. The trigger upregulates production of p-ANCA. Then, the large number of antibodies make it more likely that they will bind a neutrophil. Once bound, the neutrophil degranulates. The degranulation releases toxins that cause endothelial injury.^[6] Most recently, two different groups of investigators have demonstrated that anti-MPO antibodies alone can cause necrotizing and crescentic glomerulonephritis.^[7]

§Treatment

The customary treatment involves long term dosage of prednisone, alternated or combined with cytotoxic drugs, such as cyclophosphamide or azathioprine.

Plasmapheresis may also be indicated in the acute setting to remove ANCA antibodies.

Rituximab has been investigated,^[8] and in April 2011 approved by the FDA when used in combination with glucocorticoids in adult patients.^[2]

§See also

ANCA-associated vasculitides
Polyarteritis nodosa
List of cutaneous conditions

§References

^ ^a ^b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ ^a ^b "FDA approves Rituxan to treat two rare disorders" (Press release). Food and Drug Administration. 19 April 2011. Retrieved 20 April 2011.
"RITUXAN (rituximab) injection, solution". Daily Med. U.S. National Library of Medicine.
^ Altaie R, Ditizio F, Fahy GT (March 2005). "Microscopic polyangitis presenting with sub-acute reversible optic neuropathy". Eye (Lond) 19 (3): 363–5. doi:10.1038/sj.eye.6701479. PMID 15272290.
^ Nagai Y, Hasegawa M, Igarashi N, Tanaka S, Yamanaka M, Ishikawa O (December 2008). "Cutaneous manifestations and histological features of microscopic polyangiitis". Eur J Dermatol 19 (1): 57–60. doi:10.1684/ejd.2008.0566. PMID 19059827.
^ Seishima M, Oyama Z, Oda M (2004). "Skin eruptions associated with microscopic polyangiitis". Eur J Dermatol 14 (4): 255–8. PMID 15319159.
^ Xiao H, Heeringa P, Hu P, et al. (October 2002). "Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice". J. Clin. Invest. 110 (7): 955–63. doi:10.1172/JCI15918. PMC 151154. PMID 12370273.
^ Falk RJ, Jennette JC (July 2002). "ANCA are pathogenic—oh yes they are!". J. Am. Soc. Nephrol. 13 (7): 1977–9. PMID 12089397.
^ Jayne D (January 2008). "Challenges in the management of microscopic polyangiitis: past, present and future". Curr Opin Rheumatol 20 (1): 3–9. doi:10.1097/BOR.0b013e3282f370d1. PMID 18281850.

§External links

Overview at Johns Hopkins
Overview at Cleveland Clinic

UpToDate Contents

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1. 多発血管炎性肉芽腫症（ウェゲナー肉芽腫症）および顕微鏡的多発血管炎を伴う肉芽腫症の臨床症状および診断 clinical manifestations and diagnosis of granulomatosis with polyangiitis wegeners and microscopic polyangiitis
2. 多発血管炎性肉芽腫症（ウェゲナー肉芽腫症）および顕微鏡的多発血管炎を伴う肉芽腫症における初期免疫抑制療法 initial immunosuppressive therapy in granulomatosis with polyangiitis wegeners and microscopic polyangiitis
3. 多発血管炎性肉芽腫症（ウェゲナー肉芽腫症）および顕微鏡的多発血管炎を伴う肉芽腫症における維持免疫抑制療法 maintenance immunosuppressive therapy in granulomatosis with polyangiitis wegeners and microscopic polyangiitis
4. 多発血管炎性肉芽腫症（ウェゲナー肉芽腫症）および顕微鏡的多発血管炎を伴う肉芽腫症における患者および腎臓の転帰 patient and renal outcomes in granulomatosis with polyangiitis wegeners and microscopic polyangiitis
5. 成人の血管炎の分類とそれに対するアプローチ classification of and approach to the vasculitides in adults

English Journal

Computed Tomography Angiography in the Diagnosis of ANCA-Associated Small- and Medium-Vessel Vasculitis.

Dhaun N, Patel D, Kluth DC.SourceCentre for Cardiovascular Science, University of Edinburgh, Edinburgh, United Kingdom; Department of Renal Medicine, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom. Electronic address: bean.dhaun@ed.ac.uk.
American journal of kidney diseases : the official journal of the National Kidney Foundation.Am J Kidney Dis.2013 Apr 9. pii: S0272-6386(13)00568-4. doi: 10.1053/j.ajkd.2012.12.035. [Epub ahead of print]
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically occurs without detectable antineutrophil cytoplasmic antibody. It leads to aneurysm formation by affecting muscular arteries, usually those of medium size but also occasionally those of small size. Kidney involvement is c
PMID 23582108

Peripheral neuropathy as initial manifestation of primary systemic vasculitides.

Wolf J, Schmitt V, Palm F, Grau AJ, Bergner R.SourceDepartment of Neurology, Klinikum der Stadt Ludwigshafen, Bremserstraße 79, 67063, Ludwigshafen, Germany, wolfj@klilu.de.
Journal of neurology.J Neurol.2013 Apr;260(4):1061-70. doi: 10.1007/s00415-012-6760-7. Epub 2012 Dec 2.
Peripheral neuropathies are well-known complications of primary systemic vasculitides. In rare cases, peripheral neuropathies are among the first symptoms of these diseases. In this prospective study, 89 consecutive adult patients with newly diagnosed primary systemic vasculitis were screened, of wh
PMID 23212754

'In one ear and out the other - it's a lot to take in': a qualitative study exploring the informational needs of patients with ANCA-associated vasculitis.

Mooney J, Poland F, Spalding N, Scott DG, Watts RA.SourceSchool of Nursing Sciences, University of East Anglia, Norwich, UK. j.mooney@uea.ac.uk
Musculoskeletal care.Musculoskeletal Care.2013 Mar;11(1):51-9. doi: 10.1002/msc.1030. Epub 2012 Jul 9.
OBJECTIVES: To explore the informational needs of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV).METHODS: Three focus groups and eight one-to-one interviews were conducted with patients with AAV. A purposeful sample of 15 patients (ten female) [disease: three Churg-St
PMID 22778039

Japanese Journal

滲出性中耳炎で発症したANCA陽性の難治性中耳炎の3例

Otology Japan 24(2), 106-112, 2014
NAID 130005085319

Current Concept and Epidemiology of Systemic Vasculitides

Allergology International 63(4), 505-513, 2014
NAID 130004705230

ANCA関連血管炎にともなって眼窩先端症候群を呈した1例

臨床神経学 54(2), 158-161, 2014
NAID 130004505564

Related Pictures

★リンクテーブル★

リンク元	「顕微鏡的多発血管炎」
関連記事	「microscopic」「polyarteritis」

「顕微鏡的多発血管炎」

Microscopic polyangiitis
Classification and external resources
ICD-10	M31.7
ICD-9	446.0
DiseasesDB	8193
eMedicine	med/2931

　　[★]

英: microscopic polyangiitis, MPA
同: 顕微鏡的多発性血管炎、顕微鏡的多発動脈炎 microscopic polyarteritis
関: 血管炎。MPO-ANCA、ANCA関連血管炎

http://www.nanbyou.or.jp/sikkan/025_2_i.htm

概念

古典的なクスマウル・マイアー型(Kussmaul-Maier)の多発動脈炎に対し、分節状壊死性糸球体腎炎のような微小血管炎を伴う多発動脈炎を顕微鏡型と称したのに由来する。
小血管の炎症であるので、小血管が集まった臓器が冒される、腎臓(急速進行型糸球体腎炎 RPGN)、肺(間質性肺炎)

定義

CHCC 2012

免疫沈着を全くまたはほとんど認めない壊死性血管炎。主に小型血管(すなわち毛細血管、細静脈、または細動脈)を侵す。壊死性血管炎は小型動脈や中型動脈にも認めることがある。壊死性糸球体腎炎は非常によくみられる。肺毛細血管炎もしばしば生じる。肉芽腫性炎症は認めない。

症状

全身：発熱、体重減少、易疲労、関節痛、筋肉痛
肺　：間質性肺炎、肺出血、肺線維症
腎　：壊死性半月体形成性糸球体腎炎による急速進行性腎炎症候群(PRGN)
皮膚：紫斑、皮膚潰瘍、網状皮斑、皮下結節
神経：多発性単神経炎
消化器：消化管出血

https://www.nanbyou.or.jp/entry/245

全身症状	約70％
高血圧	約30％
皮疹	約60％
多発性単神経炎	約60％
関節痛	約50％
筋痛	約50％
間質性肺炎	約25％
肺胞出血	約10％
心不全	約18％
消化管病変	約20％

検査

尿検査

PRGNとしての所見(血尿、蛋白尿、円柱、週単位での腎機能低下)
MPO-ANCA p-ANCA 陽性　85%

肺胞洗浄液

ヘモジデリン貪食マクロファージ

治療

ANCA関連血管炎診療ガイドライン2017

https://minds.jcqhc.or.jp/n/med/4/med0094/G0000931

糖質ステロイド(GC)
シクロホスファミド(CY)/静注シクロホスファミドパルス(IVCY)/経口シクロホスファミド(POCY)
メトトレキサート(MTX)
リツキシマブ(RTX)
ミコフェノール酸モフェチル(MMF)
血漿交換

ベースとなるのはステロイドであるが、単独で使われるより、組み合わせて使われていることが多い。
どの治療が最適化は議論中のようである。

GC

GC+POCY/IVCY

GC+RTX

GC+MTX

GC+MMF

国試

112A023

			症状	検査	病理	治療
結節性多発動脈炎	polyarteritis nodosa,PN	細動脈に壊死性血管炎を引き起こす。糸球体腎炎なし。	① 全身症状あり ② 他臓器の虚血障害〈脳出血、肺出血、虚血性心疾患（心臓の冠動脈が虚血）〉 ③ 進行性腎機能低下、腎血管性高血圧(炎症動脈狭窄→レニン分泌)	尿所見に乏しい進行性腎機能低下、腰痛	・腎臓を含む多臓器の動脈に炎症が生じる。・腎動脈造影で弓状動脈に生じた結節様病変、糸球体病変は軽度。	ステロイド＆免疫抑制剤(シクロホスファミド)
顕微鏡的多発動脈炎	microscopic polyangitis,MPA	小血管の炎症。糸球体腎炎あり。	①全身症状：発熱、体重減少、多発関節炎、筋肉痛 ②多臓器の虚血障害：肺出血(血痰) ③進行性腎機能低下(急性進行性糸球体腎炎)(高齢者のRPGNにMPAが多い)	①RPGN症状が（血尿、蛋白尿、円柱、週単位での腎機能低下） ②MPO-ANCA陽性が85%を占める。	腎の微小血管と糸球体及び、肺の微小血管に炎症が生じる。 ① 半月体形成：糸球体係蹄壁の外側に増殖した細胞が半月状の形態をとる。 ② 免疫グロブリンや補体の沈着はなし。（ｐ－ＡＮＣＡがＥＬＩＺＡ法で検出される。）

「microscopic」

　　[★]

adj.

(方法)顕微鏡の、顕微鏡的な、光顕的な、(観点)顕微鏡レベルの、微視的な、顕微の

関: light-microscopic、light-microscopical、microscope、microscopical、microscopically、microscopy

「polyarteritis」

　　[★]

多発動脈炎、多発性動脈炎　

[Bolognia-1] Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.

[FDA20110419-2] "FDA approves Rituxan to treat two rare disorders" (Press release). Food and Drug Administration. 19 April 2011. Retrieved 20 April 2011.
"RITUXAN (rituximab) injection, solution". Daily Med. U.S. National Library of Medicine.

[3] Altaie R, Ditizio F, Fahy GT (March 2005). "Microscopic polyangitis presenting with sub-acute reversible optic neuropathy". Eye (Lond) 19 (3): 363–5. doi:10.1038/sj.eye.6701479. PMID 15272290.

[pmid19059827-4] Nagai Y, Hasegawa M, Igarashi N, Tanaka S, Yamanaka M, Ishikawa O (December 2008). "Cutaneous manifestations and histological features of microscopic polyangiitis". Eur J Dermatol 19 (1): 57–60. doi:10.1684/ejd.2008.0566. PMID 19059827.

[pmid15319159-5] Seishima M, Oyama Z, Oda M (2004). "Skin eruptions associated with microscopic polyangiitis". Eur J Dermatol 14 (4): 255–8. PMID 15319159.

[pmid12370273-6] Xiao H, Heeringa P, Hu P, et al. (October 2002). "Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice". J. Clin. Invest. 110 (7): 955–63. doi:10.1172/JCI15918. PMC 151154. PMID 12370273.

[7] Falk RJ, Jennette JC (July 2002). "ANCA are pathogenic—oh yes they are!". J. Am. Soc. Nephrol. 13 (7): 1977–9. PMID 12089397.

[pmid18281850-8] Jayne D (January 2008). "Challenges in the management of microscopic polyangiitis: past, present and future". Curr Opin Rheumatol 20 (1): 3–9. doi:10.1097/BOR.0b013e3282f370d1. PMID 18281850.

匿名

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案内

案内

microscopic polyarteritis