anti-glomerular basement membrane disease

抗糸球体基底膜抗体病

WordNet

1. an impairment of health or a condition of abnormal functioning
2. the lowermost portion of a structure partly or wholly below ground level; often used for storage (同)cellar
3. the ground floor facade or interior in Renaissance architecture
4. not in favor of (an action or proposal etc.)
5. a person who is opposed (to an action or policy or practice etc.); "the antis smelled victory after a long battle"
6. social insect living in organized colonies; characteristically the males and fertile queen have wings during breeding season; wingless sterile females are the workers (同)emmet, pismire
7. a thin pliable sheet of material
8. a pliable sheet of tissue that covers or lines or connects the organs or cells of animals or plants (同)tissue layer

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. 地階,地下室
4. 《話》(特定の慣習・政策・行動などに)反対する人
5. 『アリ』
6. =ain't
7. (生物の)膜,皮膜

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/08/13 18:55:02」(JST)

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Goodpasture syndrome
Classification and external resources
Micrograph of a crescentic glomerulonephritis that was shown to be anti-glomerular basement membrane disease. PAS stain.
ICD-10	M31.0 (ILDS M31.010)
ICD-9	446.21
OMIM	233450
DiseasesDB	5363
MedlinePlus	000142
eMedicine	med/923 ped/888
MeSH	D019867

Goodpasture syndrome (GPS; also known as Goodpasture’s disease, anti-glomerular basement antibody disease, or anti-GBM disease) is a rare autoimmune disease in which antibodies attack the lungs and kidneys, leading to bleeding from the lungs and to kidney failure. It may quickly result in permanent lung and kidney damage, often leading to death. It is treated with immunosuppressant drugs such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood.

The disease was first described by the American pathologist Ernest Goodpasture of Vanderbilt University, in 1919 and was later named in his honour.

Signs and symptoms

The anti-glomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalised symptoms like malaise, weight loss, fatigue, fever and chills are also common, as are joint aches and pains. 60-80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone and less than 10% have lung involvement alone. Lung symptoms usually pre-dates kidney symptoms and usually include: coughing up blood, chest pain (in less than 50% of cases overall), cough and shortness of breath. Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood and high blood pressure.

Cause

Its precise cause is unknown, but it is believed that an insult to the blood vessels taking blood from and to the lungs is required in order to allow the anti-GBM antibodies to come into contact with the alveoli. Examples of such an insult include:

• Exposure to organic solvents (e.g. chloroform) or hydrocarbons.
• Exposure to tobacco smoke.
• Certain gene mutations (HLA-DR15).
• Infection, such as influenza A.
• Cocaine inhalation.
• Metal dust inhalation.
• Bacteraemia.
• Sepsis.
• High-oxygen environments.
• Treatment with anti-lymphocytic treatment (especially monoclonal antibodies).

Pathophysiology

GPS causes the abnormal production of anti-GBM antibodies, by the plasma cells of the blood. The anti-GBM antibodies attack the alveoli and glomeruli basement membranes. These antibodies, in turn, bind their reactive epitopes to the basement membranes and activate the complement cascade, leading to the death of tagged cells. T cells are also implicated. It is generally considered a type II hypersensitivity reaction.

Diagnosis

The diagnosis of GPS is often difficult, as numerous other diseases can cause the various manifestations of the condition and the condition itself is uncommonly rare. The most accurate means of achieving the diagnosis is testing the affected tissues by means of a biopsy, especially the kidney as it is the best studied-organ for obtaining a sample, for the presence of anti-GBM antibodies. On top of the anti-GBM antibodies implicated in the disease about one in three of those affected also have cytoplasmic antineutrophilic antibodies in their bloodstream, which often pre-dates the anti-GBM antibodies by about a few months or even years. The later the disease is diagnosed the worse the outcome is for the affected person.

Treatment

The major mainstay of treatment for GPS is plasmapheresis, a procedure in which the affected person's blood is sent through a centrifuge and the various components separated based on weight. The plasma, clear liquid part of the blood, contains the anti-GBM antibodies that attacks the affected person's lungs and kidneys and is filtered out. The other parts of the blood, that is, the red blood cells, white blood cells and platelets are recycled back and given intravenously as a replacement fluid. On top of this most individuals affected by the disease need to be treated with immunosuppressants (drugs that suppress the functioning of the immune system), especially cyclophosphamide, prednisone and rituximab, to prevent the formation of new anti-GBM antibodies so as to prevent further damage to the kidneys and lungs. Other less toxic immunosuppressants like azathioprine may be used to maintain remission.

Prognosis

Without treatment virtually every affected person will end up dying from either advanced kidney failure or lung hemorrhages. With treatment the 5-year survival rate is >80% and fewer than 30% of affected individuals require long-term dialysis. Likewise the median survival time is about 5.93 years in Australia and New Zealand.

Epidemiology

GPS is rare affecting about 0.5-1.8 per million people per year in Europe and Asia. It is also unusual among autoimmune diseases in that it is more common males than in females and is also less common in blacks than whites but more common in the Maori people of New Zealand. The peak age ranges for the onset of the disease are 20-30 and 60-70 years.

See also

• HLA-DR#DR2
• Pulmonary-renal syndrome

References

External links

• GBM antibodies: immunofluorescence image

UpToDate Contents

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• 1. 抗GBM抗体（グッドパスチャー）病の病因および診断 pathogenesis and diagnosis of anti gbm antibody goodpastures disease
• 2. 抗GBM抗体（グッドパスチャー）病の治療 treatment of anti gbm antibody goodpastures disease
• 3. 抗GBM抗体病：移植後の再発 anti gbm antibody disease recurrence after transplantation
• 4. 急速進行性（半月形）糸球体腎炎の分類および治療の概要 overview of the classification and treatment of rapidly progressive crescentic glomerulonephritis
• 5. 抗好中球細胞質抗体の臨床的な意味 clinical spectrum of antineutrophil cytoplasmic antibodies

English Journal

• Rapidly progressive crescentic glomerulonephritis: Early treatment is a must.

• Moroni G1, Ponticelli C2.
• Autoimmunity reviews.Autoimmun Rev.2014 Jul;13(7):723-729. doi: 10.1016/j.autrev.2014.02.007. Epub 2014 Mar 19.
• The term crescentic glomerulonephritis (GN) refers to a pathologic condition characterized by extracapillary proliferation in >50% of glomeruli. Clinically crescentic GN is characterized by a nephritic syndrome rapidly progressing to end stage renal disease (ESRD). Three types of crescentic GN ha
• PMID 24657897

• Mode of action and clinical studies with alemtuzumab.

• Jones JL1, Coles AJ2.
• Experimental neurology.Exp Neurol.2014 May 2. pii: S0014-4886(14)00120-4. doi: 10.1016/j.expneurol.2014.04.018. [Epub ahead of print]
• The lymphocyte depleting anti-CD52 monoclonal antibody alemtuzumab has been used in Cambridge, UK, as an experimental treatment of multiple sclerosis since 1991. One phase-2 trial (CAMMS-223) and two phase-3 studies (CARE-MS1 and CARE-MS2) have confirmed its efficacy in treatment-naive patients, and
• PMID 24792641

• Membranous glomerulonephritis with crescents.

• Barrett CM1, Troxell ML, Larsen CP, Houghton DC.
• International urology and nephrology.Int Urol Nephrol.2014 May;46(5):963-71. doi: 10.1007/s11255-013-0593-x. Epub 2013 Nov 12.
• PURPOSE: The coexistence of membranous glomerulonephritis (MGN) and necrotizing and crescentic glomerulonephritis (NCGN) is an unusual finding in a renal biopsy except in lupus nephritis. Little is known about whether these lesions are causally related in any clinical setting.METHODS: We reviewed th
• PMID 24217802

Japanese Journal

• 抗糸球体基底膜抗体陽性と血栓性微小血管障害症を合併した acute kidney injury の1例

• 大久保 愛子,清水 優佳,入福 泰介,内藤 隆之,小川 貴彦,正木 崇生
• 日本透析医学会雑誌 = Journal of Japanese Society for Dialysis Therapy 45(10), 973-978, 2012-10-28
• NAID 10031130505

• 症例報告 抗糸球体基底膜抗体陽性と血栓性微小血管障害症を合併したacute kidney injuryの1例

• 大久保 愛子,清水 優佳,入福 泰介 [他]
• 日本透析医学会雑誌 45(10), 973-978, 2012-10
• NAID 40019477961

• Dense deposit disease in a 6-year-old girl

• 山田 拓司,後藤 芳充,畔柳 佳幸,田中 一樹
• Nihon Shoni Jinzobyo Gakkai Zasshi 25(2), 148-152, 2012
• … Laboratory studies revealed normal serum titers of complement and absences of anti-neutrophil cytoplasmic autoantibody (ANCA) and anti- glomerular basement membrane (GBM) antibody. … Since electron-dense deposits have been observed in the basement membrane on electron microscopy of the first renal biopsy, it was diagnosed with dense deposit disease (DDD). …
• NAID 130003346843

Related Links

• Anti-Glomerular Basement Membrane Disease

Anti-glomerular basement membrane disease (anti-GBM) is a rare but well-characterized cause of glomerulonephritis. It is defined by the presence of autoantibodies directed at specific antigenic targets within the ...

• Anti-glomerular basement membrane disease

Nephrology Forum Kidney International (2003) 64, 1535–1550; doi:10.1046/j.1523-1755.2003.00241.x Anti-glomerular basement membrane disease The Nephrology Forum is funded in part by grants from Amgen ...

Related Pictures

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★リンクテーブル★

リンク元	「血管炎」「抗糸球体基底膜抗体病」
関連記事	「disease」「glomerular」「basement」「ant」「anti」

「血管炎」

　　[★]

英

angiitis

関

脈管炎 vasculitis　vasculitides、血管内膜炎、血管炎症候群

分類

CHCC2012

large vessel vasculitis LVV 大型血管炎	Takayasu arteritis TAK 高安動脈炎
	giant cell arteritis GCA 巨細胞性動脈炎
medium vessel vasculitis MVV 中型血管炎	polyarteritis nodosa PAN 結節性多発動脈炎
	Kawasaki disease KD 川崎病
small vessel vasculitis SVV 小型血管炎	antineutrophil cytoplasmic antibody-associated vasculitis AAV 抗好中球細胞質抗体関連血管炎	microscopic polyangiitis MPA 顕微鏡的多発血管炎
		granulomatosis with polyangiitis GPA 多発血管炎性肉芽腫症(Wegener肉芽腫症)
		eosinophilic granulomatosis with polyangiitis EGPA 好酸球性多発血管炎性肉芽腫症(Churg-Strauss症候群)
	immune complex SVV 免疫複合体性小型血管炎	anti-glomerular basement membrane disease 抗糸球体基底膜抗体病(抗GBM病)
		cryoglobulinemic vasculitis CV クリオグロブリン血症性血管炎
		IgA vasculitis IgAV IgA血管炎(Henoch-Schonlein紫斑病)
		hypocomplementemic urticarial vasculitis HUV 低補体血症性蕁麻疹様血管炎(抗C1q血管炎)
variable vessel vasculitis VVV 多様な血管を侵す血管炎	Behcet's disease BD Behcet病
	Cogan's syndrome CS Cogan症候群
single-organ vasculitis SOV 単一臓器血管炎	cutaneous leukocytoclastic angiitis 皮膚白血球破砕性血管炎
	cutaneous arteritis 皮膚動脈炎
	primary central nervous system vasculitis 原発性中枢神経系血管炎
	isolated aortitis 限局性大動脈炎
vasculitis associated with systemic disease 全身性疾患関連血管炎	lupus vasculitis ループス血管炎
	rheumatoid vasculitis リウマトイド血管炎
	sarcoid vasculitis サルコイド血管炎
vasculitis associated with probable etiology 推定病因を有する血管炎	hepatitis C virus-associated cryoglobulinemic vasculitis C型肝炎ウイルス関連クリオグロブリン血症性血管炎
	hepatitis B virus-associated vasculitis B型肝炎ウイルス関連血管炎
	syphilis-associated aortitis 梅毒関連大動脈炎
	drug-associated immune complex vasculitis 薬剤関連免疫複合体性血管炎
	drug-associated ANCA-associated vasculitis 薬剤関連ANCA関連血管炎
	cancer-associated vasculitis がん関連血管炎

参考

• 血管炎症候群 - 日本内科学会雑誌第103巻第10号

• 1. 血管炎症候群の診療ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf

• 血管炎・血管障害診療ガイドライン 2016 年改訂版 - 日本皮膚科学会

https://www.dermatol.or.jp/uploads/uploads/files/vasculitisGL.pdf

「抗糸球体基底膜抗体病」

　　[★]

英

anti-glomerular basement-membrane antibody disease, anti-glomerular basement membrane disease

同

抗GBM病。グッドパスチャー症候群 Goodpasture syndrome, Goodpasture's syndrome Goodpasture症候群

関

抗糸球体基底膜疾患、実験的糸球体腎炎。anti-GBM

血管炎-小型血管炎-免疫複合体性小型血管炎

• 肺と腎が冒される
• 抗基底膜抗体によるII型アレルギー反応が起こる

概念

• 肺出血と半月体形成を伴う腎炎

• 急速進行性腎炎症候群(RPGN)を呈する

定義

CHCC 2012

• 糸球体毛細血管、肺毛細血管のいずれか一方、または双方を障害し、抗GBM抗体の沈着を伴う血管炎。肺病変は肺出血を腎病変は壊死性・半月体を伴う糸球体腎炎を引き起こす。

病因

• 抗基底膜抗体(抗糸球体基底膜抗体)が糸球体基底膜と肺胞基底膜とに反応

病理

• 基底膜に線状の抗体IgGとC3の沈着を認める(linear deposition of IgG)

• 図：BPT. Fig.14-4 B. linear, characteristic of classic anti-GBM antibody GN

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「glomerular」

　　[★]

• adj.

• 糸球体の、腎糸球体の

関

glomeruli、glomerulus、kidney glomerulus、renal glomeruli、renal glomerulus

　

「basement」

　　[★]

• n.

• 基底、地階

関

basal、bases、basis、fundus、ground

　 　 　

「ant」

　　[★]

• n.

• アリ

同

ants, stinging

　 　 　

「anti」

　　[★]

• comb form.

• 抗、アンチ