IgA糸球体腎炎
- 関
- Berger's disease、IgA nephropathy、immunoglobulin A nephropathy
WordNet
- the 9th letter of the Roman alphabet (同)i
- nephritis marked by inflammation of the glomeruli of the kidney; characterized by decreased production of urine and by the presence of blood and protein in the urine and by edema
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English Journal
- Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report.
- Gao B, Li M, Xia W, Wen Y, Qu Z.AbstractAnti-glomerular basement membrane (anti-GBM) disease is characterized by autoantibodies against antigenic site on type IV collagen of the GBM. The coexistence of anti-GBM disease and other immune complex mediated glomerulonephritis is common. Herein, we describe a patient presented with rapidly progressive glomerulonephritis, who was diagnosed as IgA-mediated nephropathy and was found to have abundant serum anti-glomerular basement membrane IgG antibodies. The patient's renal function improved considerably with intensive immunosuppressive therapy.
- Clinical nephrology.Clin Nephrol.2014 Feb;81(2):138-41. doi: 10.5414/CN107213.
- Anti-glomerular basement membrane (anti-GBM) disease is characterized by autoantibodies against antigenic site on type IV collagen of the GBM. The coexistence of anti-GBM disease and other immune complex mediated glomerulonephritis is common. Herein, we describe a patient presented with rapidly prog
- PMID 23149250
- Cytokines alter IgA1 O-glycosylation by dysregulating C1GalT1 and ST6GalNAc-II enzymes.
- Suzuki H, Raska M, Yamada K, Moldoveanu Z, Julian BA, Wyatt RJ, Tomino Y, Gharavi AG, Novak J.Author information Juntendo University, Japan;AbstractIgA nephropathy (IgAN), the most common primary glomerulonephritis, is characterized by renal immunodeposits containing IgA1 with galactose-deficient O-glycans (Gd-IgA1). These immunodeposits originate from circulating immune complexes consisting of anti-glycan antibodies bound to Gd-IgA1. As clinical disease onset and activity of IgAN often coincide with mucosal infections and dysregulation of cytokines, we hypothesized that cytokines may affect IgA1 O-glycosylation. We used IgA1-secreting cells derived from the circulation of IgAN patients and healthy controls and assessed whether IgA1 O-glycosylation is altered by cytokines. Of the eight cytokines tested, only IL-6 and, to a lesser degree, IL-4 significantly increased galactose deficiency of IgA1; changes in IgA1 O-glycosylation were robust for the cells from IgAN patients. These cytokines reduced galactosylation of the O-glycan substrate directly via decreased expression of the galactosyltransferase C1GalT1 and, indirectly, via increased expression of the sialyltransferase ST6GalNAc-II, which prevents galactosylation by C1GalT1. These findings were confirmed by siRNA knock-down of the corresponding genes and by in vitro enzyme reactions. In summary, IL-6 and IL-4 accentuated galactose deficiency of IgA1 via coordinated modulation of key glycosyltransferases. These data provide a mechanism explaining increased immune-complex formation and disease exacerbation during mucosal infections in IgAN patients.
- The Journal of biological chemistry.J Biol Chem.2014 Jan 7. [Epub ahead of print]
- IgA nephropathy (IgAN), the most common primary glomerulonephritis, is characterized by renal immunodeposits containing IgA1 with galactose-deficient O-glycans (Gd-IgA1). These immunodeposits originate from circulating immune complexes consisting of anti-glycan antibodies bound to Gd-IgA1. As clinic
- PMID 24398680
- The incidence of biopsy-proven IgA nephropathy is associated with multiple socioeconomic deprivation.
- McQuarrie EP1, Mackinnon B1, McNeice V2, Fox JG1, Geddes CC1.Author information 1Glasgow Renal and Transplant Unit, Western Infirmaryon behalf of the Scottish Renal Biopsy Registry, Glasgow, UK.2Glasgow Centre for Population Health, Glasgow, UK.AbstractChronic kidney disease is more common in areas of socioeconomic deprivation, but the relationship with the incidence and diagnosis of biopsy-proven renal disease is unknown. In order to study this, all consecutive adult patients undergoing renal biopsy in West and Central Scotland over an 11-year period were prospectively analyzed for demographics, indication, and histologic diagnosis. Using the Scottish Index of Multiple Deprivation, 1555 eligible patients were separated into quintiles of socioeconomic deprivation according to postcode. Patients in the most deprived quintile were significantly more likely to undergo biopsy compared with patients from less deprived areas (109.5 compared to 95.9 per million population/year). Biopsy indications were significantly more likely to be nephrotic syndrome, or significant proteinuria without renal impairment. Patients in the most deprived quintile were significantly more likely to have glomerulonephritis. There was a significant twofold increase in the diagnosis of IgA nephropathy in the patients residing in the most compared with the least deprived postcodes not explained by the demographics of the underlying population. Thus, patients from areas of socioeconomic deprivation in West and Central Scotland are significantly more likely to undergo native renal biopsy and have a higher prevalence of IgA nephropathy.
- Kidney international.Kidney Int.2014 Jan;85(1):198-203. doi: 10.1038/ki.2013.329. Epub 2013 Sep 11.
- Chronic kidney disease is more common in areas of socioeconomic deprivation, but the relationship with the incidence and diagnosis of biopsy-proven renal disease is unknown. In order to study this, all consecutive adult patients undergoing renal biopsy in West and Central Scotland over an 11-year pe
- PMID 24025641
Japanese Journal
- IgA腎症と細胞性半月体を伴う管内増殖性糸球体腎炎合併例に対する血漿交換とステロイドパルス療法
- 菊池 麻美/小川 哲也/樋口 千恵子/大塚 邦明,小川 哲也,樋口 千恵子 [他],大塚 邦明,KIKUCHI Asami,OGAWA Tetsuya,HIGUCHI Chieko,OTSUKA Kuniaki
- 東京女子医科大学雑誌 82(4), 229-232, 2012-08-25
- … 臨床的には高血圧,浮腫,低補体血症を認めなかったため重度のIgA腎症を疑い,第24病日に腎生検を施行した。 … 蛍光抗体法でIgAとC3のメサンギウムへの沈着を認めたが,光学顕微鏡ではメサンギウム細胞の増生と基質の増加をほとんど認めず,20%の半月体形成を伴った管内増殖性腎炎像を呈していた。 …
- NAID 110009557322
- MSSA感染を契機にネフローゼ症候群と急速進行性腎炎を呈した1例
- 桑江 紀子,謝花 政秀,宮里 朝矩 [他],和気 亨
- The Japanese journal of nephrology 54(4), 543-549, 2012-05-25
- NAID 10030739670
- The incidence and distribution of biopsy-proven renal diseases in children
- Nakashima Yumiko,Nazneen Arifa,Abe Kuniko,Nakayama Toshiyuki,Taguchi Takashi,Shirakawa Toshihiko,Moriuchi Hiroyuki
- Acta Medica Nagasakiensia 57(1), 19-24, 2012-04-00
- … Among the total biopsycases, the most common renal disease identified in childhood was IgA glomerulonephritis (IgAGN; … One of the major causes of pediatric nephrotic syndrome wereminimal change nephrotic syndrome (58%), whereas the other causes were IgAGN (11.6%), HSPN (8.9%), membranousglomerulonephritis (5.4%), membranoproliferative glomerulonephritis (3.6%). …
- NAID 110009226245
Related Links
- IgA Glomerulonephritis symptoms, causes, diagnosis, and treatment information for IgA Glomerulonephritis (IgA nephropathy) with alternative diagnoses, full-text book chapters, misdiagnosis, research treatments, prevention, and ...
- IgA Glomerulonephritis: Symptoms Incidence, Prevalence & Riskfactors Laboratory, Imaging & ECG Complications and Differential diagnoses on Symptoma®, the medical search engine for physicians. ... Acute tubulointerstitial ...
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- 英
- IgA nephropathy, immunoglobulin A nephrophathy
- 同
- IgAメサンギウム腎症 IgA mesangial nephropathy、免疫グロブリンA腎症 イムノグロブリンA腎症 immunoglobulin A nephropathy、IgA腎炎 IgA nephritis、IgA糸球体腎炎 IgA glomerulonephritis、Berger's disease、バージャー病 ベルガー病 ベルジェ病 Berger's disease Berger disease Berger病 Berger nephropathy
- 関
- 一次性糸球体疾患。
[show details]
- first aid step1 2006 p.385(Glomerular pathology)
定義
- → 同様の症状を示す、紫斑病性腎炎(Henoch-Schonlein purpura)、肝硬変、肺疾患とは区別する!!
疫学
- 日本-原発性糸球体腎炎の50-70% (約40%とする文献もある)。フランス-原発性糸球体腎炎の約20%
- 発症年齢:小児から成人まで認められる。20歳代に好発。男性が若干多い(男女比 3-6:1)
- 若年の新規透析患者の原因(約40%)として重要である。 → 透析導入原疾患の第2位
- 慢性糸球体腎炎のなかで最多(約半数)。
病因
- 免疫複合体型腎炎
- IgAを含む免疫複合体が糸球体に沈着し → 補体を活性化 → 腎炎
- 抗原:細菌、ウイルス、食物蛋白など
- begin as an episode of gross hematuria that occurs within 1 or 2 days of a nonspecific upper respiratory tract infection
病態
- 抗原と結合したIgAからなる免疫複合体が糸球体内皮下とメサンギウム領域に蓄積し腎炎像がみられる。(PRE.245) → 炎症により部分的に糸球体が破綻し血尿をきたす?
- 糸球体腎炎症状が主であって、蛋白尿は軽度。ネフローゼに至ることは稀
病理
- メサンギウム増殖性糸球体腎炎の像が見られる。
- 光学顕微鏡:メサンギウム増殖
- 蛍光顕微鏡:IgAが顆粒状にメサンギウムに沈着。C3, IgG, IgMの沈着が見られることがある
[show details]
メサンギウムにIgAが沈着する疾患
検査
- 蛋白尿は血尿に比べ軽度 ← ネフローゼ優位ではない?
診断
鑑別診断
治療
- 参考2
- 1. ACE阻害薬、アンジオテンシン受容体阻害薬
- 2. 糖質コルチコイド
- 3. 免疫抑制薬
- SPE.596
- 組織障害が軽い例:抗血小板薬、柴苓湯、ACE阻害薬、ARB
- 組織障害が強い例:副腎皮質ステロイド、抗凝固薬、抗血小板薬、免疫抑制薬の組み合わせ。
予後
- 著明な蛋白尿や腎機能低下をきたさない患者は完全に寛解するかもしれないが、ほとんどの患者ではゆっくり着実に進行する。
- 蛋白尿の持続 ± 高血圧 ± 血清Crの上昇 を下している患者は20年の経過で20-30%が末期腎不全に陥り、そのほかの20%は腎機能が低下する。
- 予後不良の腎組織像は、(稀)半月体の形成、糸球体の瘢痕化、尿細管質の萎縮、および間質の線維化である。
予後予測因子
- YN.E-43
- 血圧>160/95 mmHg、血清Cr>1.5 mg/dL、Ccr<50、尿蛋白>2g/日
予後不良因子
- 蛋白尿の持続、高血圧、高血清Cr、血清蛋白低値、高度な腎組織障害
参考
- http://www.jsn.or.jp/jsn_new/iryou/free/kousei/pdf/44_7.pdf
- 2. [charged] Treatment and prognosis of IgA nephropathy - uptodate [1]
国試
[show details]
[★]
- 英
- immunoglobulin A
- 同
- 免疫グロブリンA γA gamma A
- 関
- 免疫グロブリン Ig
概念
基準値
- 110-410 mg/dl (臨床検査法提要第32版)
臨床関連
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免疫グロブリン immunoglobulin
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糸球体腎炎 GN