急速進行性糸球体腎炎 rapidly progressive glomerulonephritis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/04/21 18:20:07」(JST)

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Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of renal function,^[1]^[2] (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months)^[2] with glomerular crescent formation seen in at least 50%^[2] or 75%^[1] of glomeruli seen on kidney biopsies. If left untreated, it rapidly progresses into acute renal failure^[3] and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus, or granulomatosis with polyangiitis; the remaining cases are idiopathic. Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).^{[medical citation needed]}

Signs and symptoms

Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome. Some patients also experience hypertension (high blood pressure) and edema. Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.^[4]

Classification

Crescentic glomerulonephritis

RPGN can be classified into three types, based upon the immunofluorescence patterns:^[5]

Type I

Accounting for approximately 20% of RPGN, type I RPGN is characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM). It is also called anti-GBM glomerulonephritis. The antibodies are directed against a particular protein found in the GBM, type IV collagen, specifically the noncollagenous region of its α₃ chain.^[4]In addition to the anti-GBM antibodies, some cases of type I RPGN are also associated with antibodies directed against the basement membrane of lung alveoli, producing Goodpasture syndrome. The majority of type I disease, however, features anti-GBM antibodies alone; these cases are considered idiopathic.^[4]

Type II

RPGN caused by the deposition of immune complexes accounts for 25% of RPGN and is classified as type II. Thus any immune complex disease that involves the glomerulus may progress to RPGN if severe enough. These diseases include systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch-Schönlein purpura, and IgA nephropathy.^[4]

Type III

Also known as pauci-immune RPGN, type III RPGN accounts for 55% of RPGN and features neither immune complex deposition nor anti-GBM antibodies. Instead, the glomeruli are damaged in an undefined manner, perhaps through the activation of neutrophils in response to ANCA. Type III RPGN may be isolated to the glomerulus (primary, or idiopathic) or associated with a systemic disease (secondary). In most cases of the latter, the systemic disease is an ANCA-associated vasculitis such as Wegener granulomatosis, microscopic polyangiitis, or Churg-Strauss syndrome.^[4]

Pathophysiology

In the pathophysiology of rapidly progressive glomerulonephritis the antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in cytoplasm of neutrophils. It is thought that ANCA causes an early degranulation giving way to release of lytic enzymes at site of injury. ANCA are linked to the pathogenesis of glomerulonephritis, antineutrophil cytoplasmic antibodies specificity is determined via (ELISA), with pANCA(antibody) directed against MPO^[6]

Diagnosis

Serum analysis often aids in the diagnosis of a specific underlying disease. The presence of anti-Glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum.^[4]

Impaired renal functions in an individual with 3 months or less of the condition is an indication of RPGN. An ultrasonographic examination of the abdomen should also be done. Upon urine examination, urinary sediment (proteinuria) can indicate proliferative glomerulonephritis, many cases of rapidly progressive glomerulonephritis need a renal biopsy to make a diagnosis.^[7]

Treatment

Therapy for rapidly progressive glomerulonephritis is done via corticosteroids and cyclophosphamide. The predictor of kidney survival is serum creatinine value. The substitution of azathioprine for cyclophosphamide after 90 day initial period is another option.Plasmapheresis can be used for patients who present with severe renal failure.^[8]

Epidemiology

The epidemiology of rapidly progressive glomerulonephritis according to Hedger,et al, is an incidence rate of 3.9 individuals per million (3.3–4.7) with a 95% confidence intervals.^[9]

References

^ ^a ^b TheFreeDictionary > rapidly progressive glomerulonephritis Citing: McGraw-Hill Concise Dictionary of Modern Medicine. 2002
^ ^a ^b ^c eMedicine > Glomerulonephritis, Crescentic Author: Malvinder S Parmar. Updated: Sep 25, 2008
^ "rapidly progressive glomerulonephritis" at Dorland's Medical Dictionary
^ ^a ^b ^c ^d ^e ^f Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, MO: Elsevier Saunders. pp. 976–8. ISBN 0-7216-0187-1.
^ Ghosh, Amit K. (2008). Mayo Clinic Internal Medicine Review: Eighth Edition (Mayo Clinic Internal Medicine Review). Informa Healthcare. p. 694. ISBN 1-4200-8478-X.
^ "Rapidly Progressive Glomerulonephritis: Background, Pathophysiology, Epidemiology".
^ Bhowmik, Dipankar (January 2011). "Clinical Approach to Rapidly Progressive Renal Failure" (PDF). Journal of the Association of Physicians of India. Retrieved October 2015.
^ "Rapidly Progressive Glomerulonephritis Treatment & Management: Medical Care, Consultations".
^ Hedger, Neil; Stevens, Judith; Drey, Nick; Walker, Sarah; Roderick, Paul (2000-10-01). "Incidence and outcome of pauci‐immune rapidly progressive glomerulonephritis in Wessex, UK: a 10‐year retrospective study". Nephrology Dialysis Transplantation 15 (10): 1593–1599. doi:10.1093/ndt/15.10.1593. ISSN 0931-0509. PMID 11007827.

Further reference

Greenhall, George H. B.; Salama, Alan D. (2015-02-19). "What is new in the management of rapidly progressive glomerulonephritis?". Clinical Kidney Journal: sfv008. doi:10.1093/ckj/sfv008. ISSN 2048-8505. PMC: 4370308. PMID 25815169.

UpToDate Contents

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1. 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
2. 小児における糸球体腎炎の病因と原因の概要 overview of the pathogenesis and causes of glomerulonephritis in children
3. 抗GBM抗体（グッドパスチャー）病の治療 treatment of anti gbm antibody goodpastures disease
4. IgA腎症の治療および予後 treatment and prognosis of iga nephropathy
5. 糸球体半月体形成の機序 mechanisms of glomerular crescent formation

English Journal

Proposal of anti-moesin as a novel biomarker for ANCA-associated vasculitis.

Suzuki K, Suzuki K, Nagao T, Nakayama T.SourceInflammation Program, Graduate School of Medicine, Chiba University, Inohana 1-8-15, Chuou-ku, Chiba, 260-8670, Japan, ksuzuki@nih.go.jp.
Clinical and experimental nephrology.Clin Exp Nephrol.2013 Sep 27. [Epub ahead of print]
Myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA) is associated with rapidly progressive glomerulonephritis (RPGN) in microscopic polyangiitis (MPA). MPO-ANCA activates neutrophils by binding to cell surface MPO expressed on tumor necrosis factor-α (TNF-α)-primed neutrophil
PMID 24072414

Correlation of interleukin-6 and monocyte chemotactic protein-1 concentrations with crescent formation and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody titer in SCG/Kj mice by treatment with anti-interleukin-6 receptor antibody or mizoribine.

Nagao T, Kusunoki R, Iwamura C, Kobayashi S, Yumura W, Kameoka Y, Nakayama T, Suzuki K.SourceInflammation Program.
Microbiology and immunology.Microbiol Immunol.2013 Sep;57(9):640-50. doi: 10.1111/1348-0421.12080.
Myeloperoxidase-specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) is associated with rapidly progressive glomerulonephritis (RPGN) and glomerular crescent formation. Pathogenic factors in RPGN were analyzed by using SCG/Kj mice, which spontaneously develop MPO-ANCA-associated RPGN. The serum
PMID 23829825

Histopathologic patterns of adult renal disease in Kermanshah, Iran: A 6-year review of two referral centers.

Mardanpour K, Rahbar M.SourceDepartment of Orthopedics, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran.
Caspian journal of internal medicine.Caspian J Intern Med.2013 Summer;4(3):717-21.
Background: The pattern of glomerular diseases in northwest Iran is unknown. This study was conducted to evaluate the histological pattern of renal diseases in this region. Methods: We retrospectively studied the reports of 266 native adult renal biopsies at the Imam Reza and Taleghani Hospitals fro
PMID 24009967

Japanese Journal

急速進行性糸球体腎炎 (AYUMI 腎炎・ネフローゼの免疫抑制療法--さらなる有効性と安全性を求めて)

臼井丈一,山縣邦弘
医学のあゆみ 237(9), 895-899, 2011-05-28
NAID 40018802185

ANCA関連血管炎による腎病変 (AYUMI ANCA関連血管炎--臓器別にみた最近の話題)

臼井丈一,山縣邦弘
医学のあゆみ 236(8), 777-782, 2011-02-19
NAID 40018267266

Related Pictures

RPGN RPGN_TRAPS.pdf」をダウンロード RPGN RPGN と RPGNから生まれた crescent from a caseof RPGN Feingewebsschnitt einer RPGN Carries poor prognosis - rapid progression 全ての糸球体ではなく巣状に

★リンクテーブル★

リンク元	「急速進行性糸球体腎炎」「MPO-ANCA」「抗糸球体基底膜抗体病」「抗好中球細胞質抗体」「まぎらわしい用語」
拡張検索	「特発性RPGN」「idiopathic RPGN」
関連記事	「RP」「RPG」

「急速進行性糸球体腎炎」

Rapidly progressive glomerulonephritis
	Histopathological image of crescentic glomerulonephritis in a patient with MPO-ANCA positive rapid progressive glomerulonephritis. Hematoxylin & eosin stain.
Classification and external resources
Specialty	Nephrology
ICD-10	N01
DiseasesDB	3165
eMedicine	med/881 med/890
	[edit on Wikidata]

　　[★]

英: rapidly progressive glomerulonephritis, RPGN
同: 急速進行性腎炎 rapidly progressive nephritis
関: 半月体形成性糸球体腎炎

まとめ

臨床的に数週から数ヶ月の経過で急速に腎機能が低下して腎不全に至る予後不良の疾患であり、病理的には半月体形成がみられる(半月体形成性糸球体腎炎)ことが特徴の疾患である。小児には稀であり、30-60歳に多く、また男性に多い(男女比=2:1)。血清学的に半月体形成性糸球体腎炎は3つの病型に分類され、それぞれ原因が異なる。type Iは抗基底膜抗体の存在が特徴的であり、特発性のものとグッドパスチャー症候群によるものがある。typeIIは免疫複合体型であり、免疫複合体の沈着が特徴的であり、特発性、感染症(溶連菌感染後急性糸球体腎炎、紫斑病性腎炎、敗血症、感染性心膜炎、B型肝炎、C型肝炎)、悪性腫瘍、自己免疫性(全身性エリテマトーデス、結節性多発性動脈炎、クリオグロブリン血症)、糸球体疾患の続発(IgA腎症、膜性増殖性糸球体腎炎)などよるものがある。type IIIはpauci-immune型(つまり蛍光顕微鏡上、免疫複合体が乏しい)でありANCAが関連しているとされ、特発性の他、ウェゲナー肉芽腫症、顕微鏡多発血管炎などが原因となる。初発症状は全身倦怠感、食欲不振、嘔気などの非特異的症状で潜行性に発症するが、急性腎炎症状やネフローゼ症候群で発症することがある。約50%の例に上気道感染症、インフルエンザ、発熱などの潜行性感染が認められる。進行すれば腎炎症状を呈し、浮腫、乏尿、腎不全、血尿・肉眼的血尿、高血圧をきたす。診断は腎生検により行う。治療は半月体が線維化する以前に(つまり細胞性)、できるだけ早期に開始する。ステロイドパルス療法、カクテル療法(副腎皮質ステロイド、免疫抑制薬、抗凝固療法、抗血小板療法)、血漿交換法を行う。予後は極めて不良であり、発症後数週から数ヶ月、遅くとも1年で腎不全に至る。(YN.E-44 PED.1247)

参考

1. 病理写真

http://www.hta-co.com/rapidly_progressive_crescentic_glomerulonephritis_02.jpg

http://www.hta-co.com/rapidly_progressive_crescentic_glomerulonephritis.jpg

nephritic syndrome

血尿、蛋白尿、乏尿、高窒素血症
乏尿がsevere

regardless of the cause, the histologic picture is characterized bu the presence of crescents.
RPGNを来すetiologyは多様
進行が急速：数週から数か月で腎不全
光顕：半月体の形成(crescentmoon shape)

「MPO-ANCA」

　　[★]

英: myeloperoxidase antineutrophil cytoplasmic antibody, myeloperoxidase-anti-neutrophil cytoplasmic antibody; perinuclear antineutrophil cytoplasmic antibody, perinuclear-anti-neutrophil cytoplasmic antibody, p-ANCA
関: PR3-ANCA=C-ANCA

鑑別疾患

顕微鏡的多発血管炎(MPA)

YN.F-62

全身の小血管に炎症を来たし、全身症状が見られるが特に肺(間質性肺炎)、腎(半月体形成性糸球体腎炎)に病変が認められる。全身症状としては発熱、体重減少、多発関節炎、筋肉痛が見られ、肺出血(血痰)、進行性腎機能低下を来たし、尿検査では血尿、蛋白尿、円柱が認められる。治療はステロイドと免疫抑制薬である。重症例では血漿交換も適応となる。死因は腎不全が多い。

アレルギー性肉芽腫性血管炎 Churg-Strauss症候群

主に中動脈以下の動脈を好発部位とする壊死性肉芽腫性血管炎。高率に肺病変と好酸球増多症を認め、また好酸球の組織浸潤も著明。気管支喘息、好酸球増加、血管炎をみとめる場合、チャーグ・ストラウス症候群である。典型的組織像が見とめられればアレルギー性肉芽腫性血管炎と呼ばれる。検査では、白血球増加、血小板数増加、血清IgE増加、MPO-ANCA陽性、リウマトイド因子陽性、肺浸潤陰影が認められる。治療はステロイドや免疫抑制薬である。結節性多発動脈炎より予後良好であるが脳血管障害、呼吸不全、腸管出血、感染が死因となりうる。