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the 16th letter of the Roman alphabet (同)p
a river in western Thailand; a major tributary of the Chao Phraya (同)Ping River

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phosphorusの化学記号
palladiumの化学記号

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/27 18:53:18」(JST)

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Perinuclear staining typical of p-ANCA

p-ANCA, or MPO-ANCA, or Perinuclear Anti-Neutrophil Cytoplasmic Antibodies, show a perinuclear staining pattern.

This pattern occurs because the vast majority of the antigens targeted by ANCAs are highly cationic (Positively charged) at pH 7.00. During ethanol (pH ~7.0 in water) fixation, antigens which are more cationic migrate and localize around the nucleus, attracted by its negatively charged DNA content. Antibody staining therefore results in fluorescence of the region around the nucleus.

TargetsEdit

By far the most common p-ANCA target is myeloperoxidase (MPO), a neutrophil granule protein whose primary role in normal metabolic processes is generation of oxygen radicals.

ANCA will less commonly form against alternative antigens that may also result in a p-ANCA pattern. These include lactoferrin; elastase; and cathepsin G.

When the condition is a vasculitis, the target is usually MPO.^[1] However, the proportion of p-ANCA sera with anti-MPO antibodies has been reported to be as low as 12%.^[2]

Medical conditionsEdit

p-ANCA is associated with several medical conditions:^[3]

It is fairly specific, but not sensitive for ulcerative colitis, so is not useful as a sole diagnostic test.^[4] When measured together with Anti-saccharomyces cerevisiae antibodies (ASCA), p-ANCA has been estimated to have a specificity of 97% and a sensitivity of 48% in differentiating patients with ulcerative colitis from normal controls.^[5]
about 50% of cases of Churg-Strauss syndrome
A majority of primary sclerosing cholangitis
sometimes polyarteritis nodosa (only in the variant microscopic polyangiitis, usually negative in typical polyarteritis nodosa)
microscopic polyangiitis ^[6]
Focal necrotising and crescentic glomerulonephritis
Rheumatoid arthritis

ReferencesEdit

^ Anthony S. Fauci; Carol A. Langford (16 March 2006). Harrison's rheumatology. McGraw-Hill Professional. pp. 159–. ISBN 978-0-07-145743-9. Retrieved 1 November 2010.
^ Yehuda Shoenfeld; M. Eric Gershwin; Pier-Luigi Meroni (2007). Autoantibodies. Elsevier. pp. 98–. ISBN 978-0-444-52763-9. Retrieved 1 November 2010.
^ Mary Lee (10 March 2009). Basic Skills in Interpreting Laboratory Data. ASHP. pp. 455–. ISBN 978-1-58528-180-0. Retrieved 15 November 2010.
^ Shepherd B, et al. (2005). "Inflammatory Bowel Disease: Diagnostic and Treatment Options". Hospital Physician: 11–19.
^ [1] Walker, D. G.; Bancil, A. S.; Williams, H. R.; Bunn, C.; Orchard, T. R. (2011). "How helpful are serological markers in differentiating crohn's disease from ulcerative colitis in indian asian inflammatory bowel disease patients?". Gut 60: A222–A223. doi:10.1136/gut.2011.239301.469.
^ Thomas M. Habermann; Mayo Clinic (1 November 2007). Mayo Clinic Internal Medicine Concise Textbook. CRC Press. pp. 775–. ISBN 978-1-4200-6749-1. Retrieved 15 November 2010.

UpToDate Contents

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1. 抗好中球細胞質抗体の臨床的な意味 clinical spectrum of antineutrophil cytoplasmic antibodies
2. 好酸球性肉芽腫性多発血管炎(チャーグ・ストラウス症候群）の臨床的特徴および診断 clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis churg strauss
3. 多発血管炎性肉芽腫症を伴う肉芽腫症および関連血管炎の病因 pathogenesis of granulomatosis with polyangiitis and related vasculitides
4. 多発血管炎性肉芽腫症（ウェゲナー肉芽腫症）における気道病変 respiratory tract involvement in granulomatosis with polyangiitis wegeners
5. 好酸球性肉芽腫性多発血管炎(チャーグ・ストラウス症候群）の疫学、病因、および病理 epidemiology pathogenesis and pathology of eosinophilic granulomatosis with polyangiitis churg strauss

English Journal

A difficult case of fever of unknown origin.

Pang CL, Richardson P, Makkuni D.SourceDepartment of Trauma and Orthopaedics, James Paget University Hospital, Norwich, UK.
BMJ case reports.BMJ Case Rep.2012 Jul 9;2012. pii: bcr1120115210. doi: 10.1136/bcr.11.2011.5210.
A 57-year-old holidaymaker in Madeira was airlifted to England with a 4-week history of fever, limb weakness and hypophonia. Having undergone a range of investigations during his hospital admission abroad - including ultrasound, CT, echocardiogram and lumbar puncture - the patient arrived without an
PMID 22778463

[Sjögren's syndrome and vasculitis associated with antineutrophil cytoplasmic antibodies.]

Lema Gontad JM, Espinosa Garriga G.SourceServicio de Enfermedades Autoinmunes Sistémicas, Institut Clínic de Medicina i Dermatologia, Hospital Clinic, Barcelona, España.
Medicina clinica.Med Clin (Barc).2012 Jul 3. [Epub ahead of print]
PMID 22766054

Japanese Journal

耳症状のみで発症したウェゲナー肉芽腫症疑い例

高岡麻里絵,萩原晃,長谷川達哉,小川恭生,西山信宏,鈴木衞
耳鼻咽喉科臨床 103(3), 201-208, 2010-03-01
… Systemic examination shared slightly elevated serum P-ANCA but negative C-ANCA. …
NAID 10026216819

耳症状で初発し肥厚性硬膜炎を合併したWegener肉芽腫症疑い例

深美悟,春名眞一,平林秀樹,月舘利治,岡田真由美,金谷洋明
Otology Japan 20(3), 173-179, 2010
… 耳症状を初発とし、C-ANCA、P-ANCA陰性で、鼓室開放術での組織検査でも特徴的所見がなく、経過中に肥厚性硬膜炎を合併したWegener肉芽腫症疑い症例を経験した。 … 経過中にC-ANCAの上昇、脳MRIで肥厚性硬膜炎を認め、肥厚性硬膜炎を伴ったWegener肉芽腫症疑い例と診断した。 …
NAID 130001471717

進行性難聴を呈したP-ANCA陽性 Wegener 肉芽腫症

坂口博史,任書晃,瀧正勝,鈴木敏弘,久育男
耳鼻咽喉科臨床 102(7), 517-522, 2009-07-01
… We report a case of P-ANCA positive Wegener’s granulomatosis (WG) with progressive hearing loss. … She was diagnosed as having WG based on specific symptoms in the upper respiratory tract (otitis and sinusitis), pathological findings in the nasal mucosa and blood test data including positive findings for P-ANCA. …
NAID 10025096537

Related Pictures

File:P ANCA .jpg Perinuclear staining typical of p-ANCA Searched Term: p anca Homogenous perinuclear Positive+P+Anca Anticorps anti-cytoplasme Los ANCA en las vasculitis ANCA positivas name: MASTAFLUOR c-Anca, p-Anca IgG Results for P Anca Disease.

★リンクテーブル★

リンク元	「原発性硬化性胆管炎」「抗好中球細胞質抗体」
関連記事	「ANCA」「AN」「P」「p」「Pd」

「原発性硬化性胆管炎」

　　[★]

英: primary sclerosing cholangitis, PSC
関: 硬化性胆管炎、胆管炎

概念

稀で原因不明の疾患。
肝内・肝外胆管に原因不明の線維性狭窄を来し、持続性あるいは再発性(反復性)の閉塞性黄疸を来す疾患。 (YN.B-73)
炎症により胆管壁の周囲に結合織が増生・硬化・狭窄し、胆管の狭窄により胆汁うっ滞による症状を呈し、慢性経過で胆管炎による胆汁うっ滞性肝硬変に進展。

病因

原因不明

自己免疫が関連？HLA-B8

疫学

20歳代、50-60歳代にピーク
男女比=2:1

遺伝形式

病理

胆管を取り巻く線維化(線維性閉塞性胆管炎)(YN.B-73)

病態

胆管壁の全長～一部に線維性の肥厚が生じて内腔が狭窄する。肝外胆管と肝内胆管の比較的太い部位に限局する(⇔原発性胆汁性肝硬変: 肝内胆管の細胆管の障害)

進行性であり、胆汁性肝硬変に至り、肝硬変や食道静脈瘤破裂により死亡。

症状

本症の7-50%は全く無症状。症状があるものでは、体重減少(35-80%)、黄疸(25-75%)、掻痒感(10-70%)、右季肋部痛・心窩部痛(75%)、全身倦怠感、発熱。(NSU.643)
閉塞性黄疸：消長を繰り返す(SSUR.615)
胆管炎：胆管の閉塞・狭窄による

検査

MRCP、ERCP、(PTC: 今ではあんまりやらない？。肝内胆管周囲の線維化のために不成功に終わる事が多い(NSU.643))

肝内・肝外胆管の限局性の狭窄と正常部が交互に現れる：びまん性の数珠状の狭窄　→　beaded appearance (NSU.643 SSUR.615)
肝内胆管の細枝が狭窄: pruned tree appearance

血液：胆道系酵素
血清学的検査：P-ANCA：陽性、AMA：90%以上の症例で陰性

診断

閉塞性黄疸の検査所見(胆道系酵素上昇、直接ビリルビン上昇)、胆道造影、血清学的検査(AMA陰性)

鑑別診断

胆管癌

合併症

YN.B-73

治療

対症療法：

副腎皮質ホルモン、免疫抑制薬、(肝庇護)ウルソデオキシコール酸、
胆道ドレナージ
胆道内瘻化術
肝外胆管切除術
胆管空腸吻合術

根治療法：(肝不全に陥った症例)肝移植

予後

肝移植後の5年生存率：85-90%(YN.B-73)
経過中に胆管癌(9-15)が発生しうる。(YN.B-73)

see BPT.659

Table 16-7. Main Features of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis
Parameter	primary biliary cirrhosis	primary sclerosing cholangitis
Age	Median age 50 years (30-70)	Median age 30 years
Gender	90% female	70% male
Clinical course	Progressive	Unpredictable but progressive
Associated conditions	Sjogren syndrome (70%)	inflammatory bowel disease (70%)
	scleroderma (5%)	pancreatitis (?25%)
	thyroid disease (20%)	idiopathic fibrosing disease (retroperitoneal fibrosis)
Serology	95% AMA positive	0% to 5% AMA positive (low titer)
	20% ANA positive	6% ANA positive
	60% ANCA positive	82% ANCA positive
Radiology	normal	strictures and beading of large bile ducts; pruning of smaller ducts
duct lesion	florid duct lesion; loss of small ducts	concentric periductal fibrosis; loss of small ducts