今日の臨床サポート

紫斑病性腎炎

著者: 佐々木彰 飯塚病院 腎臓内科/臨床研究支援室

監修: 岡田浩一 埼玉医科大学 腎臓内科

著者校正/監修レビュー済:2020/05/14
参考ガイドライン:
  1. 合同研究班参加学会:血管炎症候群の診療ガイドライン 2017年改訂版
  1. KDIGO:Clinical Practice Guideline for Glomerulonephritis 2012(初版)
患者向け説明資料

概要・推奨   

  1. IgA血管炎患者に、腎症発症予防の観点から、早期にステロイド投与を行うことは勧められない(推奨度3S/CS
  1. 軽症の患者も含んだ紫斑病性腎炎に対して、内服ステロイドの有効性については、一定の結論を得ていない(推奨度3O
  1. 重症の紫斑病性腎炎の患者では、ステロイドパルス療法を行うことで、病勢のコントロールが可能となる可能性がある(推奨度1O
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  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
佐々木彰 : 特に申告事項無し[2021年]
監修:岡田浩一 : 講演料(協和キリン,中外製薬,田辺三菱,第一三共),研究費・助成金など(協和キリン),奨学(奨励)寄付など(協和キリン,中外製薬,田辺三菱,第一三共,アステラス,MSD,武田薬品,鳥居薬品,ファイザー,ノバルティス,日本ベーリンガーインゲルハイム,大塚製薬,塩野義,大日本住友)[2021年]

改訂のポイント:
  1. 定期レビューを行った(変更なし)。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. IgA血管炎(Henoch–Schönlein purpura)とは、IgAを含む免疫複合体の沈着を全身の小血管に認め、その結果血管炎を生じる疾患である。皮膚、腸管、関節、糸球体が主に障害を受ける。
  1. 紫斑病性腎炎は、IgA血管炎の一症状としてみられる腎炎である。
  1. IgA血管炎自体は小児(4~10歳)に多く、男女比は2:1とされている。一方、成人でもIgA血管炎がみられるが、成人では腎症が重症化する頻度が高いとされている。
  1. IgA血管炎には4徴として、紫斑、関節痛、腹痛、腎障害がある。
  1. このうち腎障害は、小児では16~50%にみられるとされている。IgA血管炎の発症後1~2週から数カ月(約80%では1カ月以内)に腎症の発現を認める。
  1. 単独血尿程度から、蛋白尿を認めるもの、急性腎炎・ネフローゼを認めるものまでその程度はさまざまである。
  1. 高齢発症、発症時腎機能低下、病初期にネフローゼ症候群、血尿を伴うネフローゼ症候群を呈した例では、腎機能予後が悪いとされている。
  1. 発症時血尿のみ、軽度尿蛋白(1g/日未満)を呈した例では、腎機能予後が良いとされている。
  1. IgA腎症と同様に糖鎖構造に異常のあるIgA1が血中に増加していて、こうした糖鎖異常IgA1がメサンギウム領域に沈着しやすいことが病態の発生に関与していることが示されている。
  1. 紫斑病性腎炎は、指定難病であり、国際小児腎臓病研究班(ISKDC)による紫斑病性腎炎の組織分類でGradeIIIb以上の場合などでは、申請し認定されると保険料の自己負担分の一部が公費負担として助成される。(平成27年7月施行)
  1. 難病法に基づく医療費助成制度
 
  1. 臨床所見では、高齢発症、腎機能低下、病初期のネフローゼ症候群は腎機能予後不良と関連する発症時血尿のみであること、軽度尿蛋白(1g/日未満)であることが、それぞれ良好な腎機能予後と関連するS)。
  1. まとめ:本疾患の発症時臨床所見で、腎機能予後と関連するものが複数報告されている。
  1. 代表事例:1972年1月から2019年2月までの期間で、PubMed、Embase、Web of Scienceに収載されている研究を対象に実施された系統レビューおよびメタ解析の結果、9つの症例対照研究(内7つは質が高く、2つの質は中等度)が解析対象となった。腎機能予後不良([活動性腎臓病:高血圧または尿蛋白>40mg/m2または血清Cre上昇を含む]および[尿毒症/末期腎不全:透析または腎移植を含む])と関連する因子として、高齢発症(weighted mean difference: WMD 1.77, 95%CI 0.35-3.18, p=0.014)、糸球体濾過量低下(WMD -23.93, 95%CI -3378—14.09, p<0.0001)、発症時ネフローゼ症候群(OR 1.74, 95%CI 1.12-2.70, p=0.013)、血尿を伴うネフローゼ症候群(OR 4.55, 95%CI 2.89-7.15, p<0.0001)が示された。発症時血尿のみ(0.33 95%CI 0.16-0.69, p=0.003)、軽度尿蛋白(1g/日未満)(OR 0.46, 95%CI 0.28-0.75, p<0.0001)は良好な腎機能予後と関連していた。
  1. 結論:これまでの本疾患における腎機能予後に関する報告を統合した結果、腎機能予後不良因子として高齢発症、腎機能低下、病初期のネフローゼ症候群、腎機能予後良好因子として発症時血尿のみ、軽度尿蛋白(1g/日未満)が示された[1]
  1. 腎障害の経過はおおむね良好であるが、成人での腎機能予後は小児に比較すると不良であるO
  1. まとめ:本疾患は小児に多くみられる疾患であるが、小児ではおおむね予後は良好である。一方、成人では、小児に比較すると末期腎不全に至る頻度は高い。
  1. 代表事例:組織学的に軽症の患者も含んだ88人の紫斑病性腎炎患児について、平均9.9年観察した検討[2]では、3人(3.4%)が維持透析を必要としていた。イタリアの多施設共同観察研究でも、219人の紫斑病性腎炎(16歳未満の小児 83人、成人136人)を対象とし予後が検討されている。腎生検組織はISKDC gradeでGrade I 3.7%、II 54.3%、III 34.0%、IV 2.6%、V 0%であった。観察期間の中央値4.5年で、維持透析施行患者は小児で7.2% (6人)、 成人で 13.2% (18人)と成人で多い傾向にあった(log-rank p=0.082)。一方、クレアチンの倍化は有意に成人で多くみられた(小児 12人、成人 35人、log-rank p=0.014)[3]。フランスの11施設で腎生検を行われた15歳以上の紫斑病性腎炎患者250人を対象とした検討[4]では、14.8年の観察期間中に64人(26%)が中央値15年で死亡したが、27人(11%)が末期腎不全に至った。クレアチニンクリアランス30ml/分未満の高度腎不全の予測因子として、生検時のクレアチニン>120μmol/L (RR 4.27)、蛋白尿>1g/日 (RR 2.98)、壊死糸球体>10% (RR 1.83)、硬化糸球体>20% (RR 2.12)、間質の線維化>10% (RR 3.83)が選択された。
  1. 結論:いずれの報告でも、小児では、少なくとも末期腎不全に至る患者は比較的少なく、おおむね良好な予後が示された。一方、成人では小児に比較すると、末期腎不全に至る患者が多い傾向にあった。
問診・診察のポイント  
  1. 過去にIgA血管炎と診断されたことがないかを問診する。

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文献 

著者: Dongmei Shi, Han Chan, Xia Yang, Gaofu Zhang, Haiping Yang, Mo Wang, Qiu Li
雑誌名: PLoS One. 2019;14(10):e0223218. doi: 10.1371/journal.pone.0223218. Epub 2019 Oct 1.
Abstract/Text OBJECTIVE: To identify risk factors associated with unfavorable outcomes in children with IgA vasculitis with nephritis (Henoch-Schőnlein purpura nephritis)(IgA-VN).
METHODS: PubMed, Embase, and Web of Science databases were searched for studies, published in English through February 2019. The data were extracted to perform pooled analysis, heterogeneity testing, subgroup analysis, sensitivity analysis, and publication bias analysis.
RESULTS: This meta-analysis showed that, older age at onset (WMD 1.77, 95% CI 0.35-3.18, p = 0.014), lower glomerular filtration rate (GFR; WMD -23.93, 95% CI -33.78- -14.09, p<0.0001), initial renal manifestations with nephrotic syndrome (OR 1.74, 95% CI 1.12-2.70, p = 0.013), with nephritic-nephrotic syndrome (OR 4.55, 95% CI 2.89-7.15, p<0.0001) and renal biopsy with crescentic nephritis (International Study of Kidney Disease in Children [ISKDC] grades III-V) (OR 3.85, 95% CI 2.37-6.28, p<0.0001) were significant risk factors associated with poor outcomes in IgA-VN, whereas initial clinical features with hematuria (OR 0.33, 95% CI 0.16-0.69, p = 0.003) and mild proteinuria±hematuria (OR 0.46, 95% CI 0.28-0.75, p<0.0001) were associated with progression to good outcomes. By contrast, gender, hypertension and initial renal manifestations of acute nephritic syndrome were not significantly associated with poor outcomes in IgA-VN.
CONCLUSION: This meta-analysis showed that older age at onset, lower GFR, initial renal features of nephrotic syndrome and nephritic-nephrotic syndrome and renal biopsy with crescentic nephritis (ISKDC grades III-V) were predictive of poor prognosis in children with IgA-VN.

PMID 31574112  PLoS One. 2019;14(10):e0223218. doi: 10.1371/journal.po・・・
著者: R Counahan, M H Winterborn, R H White, J M Heaton, S R Meadow, N H Bluett, H Swetschin, J S Cameron, C Chantler
雑誌名: Br Med J. 1977 Jul 2;2(6078):11-4.
Abstract/Text All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.

PMID 871734  Br Med J. 1977 Jul 2;2(6078):11-4.
著者: Rosanna Coppo, Simeone Andrulli, Alessandro Amore, Bruno Gianoglio, Giovanni Conti, Licia Peruzzi, Francesco Locatelli, Leonardo Cagnoli
雑誌名: Am J Kidney Dis. 2006 Jun;47(6):993-1003. doi: 10.1053/j.ajkd.2006.02.178.
Abstract/Text BACKGROUND: Factors predictive of renal outcome were investigated in 219 cases of biopsy-proven Henoch-Schönlein purpura nephritis (HSPN); 83 children and 136 adults enrolled in a national study were followed up for up to 27 years (median, 4.5 years).
METHODS: The criterion for defining disease progression was time elapsed until doubling of baseline creatinine level and until dialysis therapy. Age, sex, data at onset (renal function, proteinuria, hematuria, hypertension, and crescents), and data during follow-up (proteinuria and therapy) were tested as covariates.
RESULTS: Multivariate Cox regression analysis indicated the following parameters as independent prognostic predictors: age (adults versus children, relative risk, 3.57; 95% confidence interval, 1.18 to 10.79; P = 0.024 for creatinine level doubling; relative risk, 14.89; 95% confidence interval, 1.72 to 129.07; P = 0.014 for dialysis therapy), sex (females versus males, relative risk, 5.71; 95% confidence interval, 1.67 to 19.55; P = 0.006 for creatinine level doubling; relative risk, 26.03; 95% confidence interval, 2.64 to 256.73; P = 0.005 for dialysis therapy), and mean proteinuria during follow-up (for each 1 g/d of protein increase, relative risk, 1.77; 95% confidence interval, 1.35 to 2.32; P < 0.001 for creatinine level doubling; relative risk, 1.73; 95% confidence interval, 1.18 to 2.52; P = 0.005 for dialysis therapy). Information for mean proteinuria levels during follow-up increased the sensitivity at logistic regression to 62.5%, with dialysis therapy as the end point. No data detected at diagnosis, including renal function impairment, proteinuria, hypertension, and crescentic nephritis (involving > 50% of glomeruli in only 2.6%), were significantly related to functional decline at multivariate Cox.
CONCLUSION: This analysis indicates that, even more than when decreased renal function, severe proteinuria, hypertension, or crescents are present at onset, the risk for progression of HSPN (greater in adults and females) was associated with increasing mean proteinuria levels during follow-up.

PMID 16731294  Am J Kidney Dis. 2006 Jun;47(6):993-1003. doi: 10.1053/・・・
著者: Evangéline Pillebout, Eric Thervet, Gary Hill, Corinne Alberti, Philippe Vanhille, Dominique Nochy
雑誌名: J Am Soc Nephrol. 2002 May;13(5):1271-8.
Abstract/Text Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.

PMID 11961015  J Am Soc Nephrol. 2002 May;13(5):1271-8.
著者: J C Davin, I J Ten Berge, J J Weening
雑誌名: Kidney Int. 2001 Mar;59(3):823-34. doi: 10.1046/j.1523-1755.2001.059003823.x.
Abstract/Text Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are considered to be related diseases since both can be encountered consecutively in the same patient, they have been described in twins, and bear identical pathological and biological abnormalities. Apart from the presence of extrarenal clinical signs found only in HSPN, other differences are noticed between the two diseases. The peak age ranges between 15 and 30 years for a diagnosis of IgAN, whereas HSPN is mainly seen in childhood. Nephritic and/or nephrotic syndromes are more often seen at presentation in HSPN. In contrast to IgAN, HSPN has been described in association with hypersensitivity. Endocapillary and extracapillary inflammations as well as fibrin deposits in the glomerulus are more frequent in HSPN. No major biological differences have been found between the two illnesses, except for a larger size of circulating IgA-containing complexes (IgA-CC) and a greater incidence of increased plasma IgE levels in HSPN. As tissue infiltration by leukocytes is a major feature of HSPN vasculitis, a possible role of a more potent activation of the latter cells by IgA-CC and/or circulating chemokines in HSPN should be considered. Further studies are required to elucidate this possible mechanism as well as the role of hypersensitivity in HSPN.

PMID 11231337  Kidney Int. 2001 Mar;59(3):823-34. doi: 10.1046/j.1523-・・・
著者: Hiroyuki Komatsu, Shouichi Fujimoto, Norishige Yoshikawa, Hiroshi Kitamura, Hitoshi Sugiyama, Hitoshi Yokoyama
雑誌名: Clin Exp Nephrol. 2016 Aug;20(4):552-60. doi: 10.1007/s10157-015-1177-0. Epub 2015 Oct 11.
Abstract/Text BACKGROUND: The clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) has not been thoroughly investigated among patients of different ages. We therefore compared the features of HSPN and IgA nephropathy (IgAN) based on data from the Japan Renal Biopsy Registry (J-RBR).
METHODS: This cross-sectional study analyzed data from patients who were registered in the J-RBR between 2007 and 2012. Clinico-pathological findings at diagnosis were compared among children (aged ≤18 years), adult (aged 19-64 years) and elderly (aged ≥65 years) patients with HSPN (n = 513) and IgAN (n = 5679).
RESULTS: The age at diagnosis considerably differed between HSPN and IgAN; HSPN peaked at 1-19 and at 60-69 years, whereas IgAN peaked at 30-39 years. The clinical features were significantly more severe for HSPN than IgAN, especially proteinuria (children, 1.28 vs. 0.57; adult, 1.95 vs. 1.05; elderly patients, 2.71 vs. 1.64 g/day), and low albumin levels (children, 3.72 vs. 4.13; adults, 3.62 vs. 3.99; elderly patients, 3.07 vs. 3.57 g/dL). The rate (%) of histologically classified endocapillary proliferative or crescentic glomerulonephritis was higher in patients with HSPN than with IgAN. Multiple regression analysis revealed that low albumin level and high BP were independent factors associated with decreased estimated glomerular filtration rates in adult and elderly patients with HSPN.
CONCLUSIONS: Age at HSPN diagnosis was bimodally distributed, and the clinical features of HSPN were more severe than those of IgAN across all age groups.

PMID 26456327  Clin Exp Nephrol. 2016 Aug;20(4):552-60. doi: 10.1007/s・・・
著者: A R Goldstein, R H White, R Akuse, C Chantler
雑誌名: Lancet. 1992 Feb 1;339(8788):280-2.
Abstract/Text A study of long-term outcome of 78 subjects who had had Henoch-Schönlein nephritis during childhood (at a mean of 23.4 years after onset) shows that severity of clinical presentation and initial findings on renal biopsy correlate well with outcome but have poor predictive value in individuals. 44% of patients who had nephritic, nephrotic, or nephritic/nephrotic syndromes at onset have hypertension or impaired renal function, whereas 82% of those who presented with haematuria (with or without proteinuria) are normal. 17 patients deteriorated clinically from an initial assessment in 1971; 7 of these had apparently completely recovered in 1976. 16 of 44 full-term pregnancies were complicated by proteinuria and/or hypertension, even in the absence of active renal disease. These findings indicate that childhood Henoch-Schönlein nephritis requires long-term follow-up, especially during pregnancy.

PMID 1346291  Lancet. 1992 Feb 1;339(8788):280-2.
著者: Pamela F Weiss, James A Feinstein, Xianqun Luan, Jon M Burnham, Chris Feudtner
雑誌名: Pediatrics. 2007 Nov;120(5):1079-87. doi: 10.1542/peds.2007-0667.
Abstract/Text OBJECTIVE: No consensus exists among general pediatricians or pediatric rheumatologists regarding whether corticosteroid therapy ameliorates the acute manifestations of Henoch-Schönlein purpura or mitigates renal injury. Therefore, we sought to synthesize the reported experimental and observational data regarding corticosteroid use.
METHODS: We performed a meta-analysis based on a comprehensive review of the literature in the Medline database (1956 to January 2007) and the Cochrane Controlled Trials Register. On the basis of reported outcomes among patients with Henoch-Schönlein purpura who were treated at diagnosis with corticosteroids compared with patients treated with supportive care only, we calculated odds ratios for the resolution of abdominal pain, the need for surgical intervention secondary to severe pain or intussusception, the likelihood of Henoch-Schönlein purpura recurrence, and the development of transient or persistent renal disease.
RESULTS: Of 201 articles retrieved from the initial literature search, 15 were eligible for inclusion. Corticosteroid treatment did not reduce the median time to resolution of abdominal pain but did significantly reduce the mean resolution time and increased the odds of resolution within 24 hours. Early corticosteroid treatment significantly reduced the odds of developing persistent renal disease. In addition, although the results were not statistically significant, the prospective data suggest reduced odds of both surgical intervention and recurrence.
CONCLUSIONS: Corticosteroids, given early in the course of illness, seem to produce consistent benefits for several major clinically relevant Henoch-Schönlein purpura outcomes.

PMID 17974746  Pediatrics. 2007 Nov;120(5):1079-87. doi: 10.1542/peds.・・・
著者: W Chartapisak, S Opastiraku, N S Willis, J C Craig, E M Hodson
雑誌名: Arch Dis Child. 2009 Feb;94(2):132-7. doi: 10.1136/adc.2008.141820. Epub 2008 Aug 13.
Abstract/Text OBJECTIVE: To determine the benefits and harms of therapies used to prevent or treat renal involvement in Henoch-Schönlein purpura.
DESIGN: Systematic review of randomised controlled trials.
SETTING: Secondary and tertiary paediatric and paediatric nephrology services.
SUBJECTS: Ten trials involving 1230 children aged less than 18 years.
MAIN OUTCOME MEASURES: Persistent proteinuria and/or haematuria.
RESULTS: Meta-analyses of four trials showed no significant difference in the risk of persistent kidney disease at 6 months (379 children; relative risk (RR) 0.51, 95% CI 0.24 to 1.11) and 12 months (498 children; RR 1.02, 95% CI 0.40 to 2.62) in children given prednisone for 14-28 days at presentation of Henoch-Schönlein purpura compared with placebo or supportive treatment. In children with severe renal disease, there was no significant difference in the risk of persistent renal disease with cyclophosphamide compared with supportive treatment (one trial; 56 children; RR 1.07, 95% CI 0.65 to 1.78) and with cyclosporin compared with methylprednisolone (one trial; 19 children; RR 0.39; 95% CI 0.14 to 1.06).
CONCLUSIONS: Data from randomised trials for any intervention used to improve renal outcomes in children with Henoch-Schönlein purpura are very sparse except for short-term prednisone, which has not been shown to be effective.

PMID 18701559  Arch Dis Child. 2009 Feb;94(2):132-7. doi: 10.1136/adc.・・・
著者: Wattana Chartapisak, Sauwalak Opastirakul, Elisabeth M Hodson, Narelle S Willis, Jonathan C Craig
雑誌名: Cochrane Database Syst Rev. 2009 Jul 8;(3):CD005128. doi: 10.1002/14651858.CD005128.pub2. Epub 2009 Jul 8.
Abstract/Text BACKGROUND: To determine the benefits and harms of therapies used to prevent or treat kidney disease in Henoch-Schönlein Purpura (HSP).
OBJECTIVES: To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo or no treatment or another agent for the prevention or treatment of kidney disease in patients with HSP.
SEARCH STRATEGY: Randomised controlled trials (RCTs) and quasi-RCTs were identified from the Cochrane Renal Group's specialised register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE using optimally sensitive search strategies combined with search terms for HSP.
SELECTION CRITERIA: RCTs comparing any intervention used to prevent or treat kidney disease in HSP compared with placebo, no treatment or other agents were included.
DATA COLLECTION AND ANALYSIS: Three authors independently assessed trial quality and extracted data from each study. Statistical analyses were performed using the random effects model and the results were expressed as risk ratio (RR) for dichotomous outcomes and mean difference (MD) for continuous outcomes with 95% confidence intervals (CI).
MAIN RESULTS: Ten studies (1230 children) were identified. There was no significant difference in the risk of persistent kidney disease at six months (3 studies, 379 children: RR 0.51, 95% CI 0.24 to 1.11) and 12 months (3 studies, 498 children: RR 1.02, 95% CI 0.40 to 2.62) in children given prednisone for 14 to 28 days at presentation of HSP compared with placebo or supportive treatment. In children with severe kidney disease, there was no significant difference in the risk of persistent kidney disease with cyclophosphamide compared with supportive treatment (1 study, 56 children: RR 1.07, 95% CI 0.65 to 1.78) and with cyclosporin compared with methylprednisolone (1 study, 19 children: RR 0.39, 95% CI 0.14 to 1.06).
AUTHORS' CONCLUSIONS: Data from RCTs for any intervention used in improve kidney outcomes in children with HSP are very sparse except for short-term prednisone. There was no evidence of benefit of prednisone in preventing serious long-term kidney disease in HSP.

PMID 19588365  Cochrane Database Syst Rev. 2009 Jul 8;(3):CD005128. do・・・
著者: Jean-Claude Davin
雑誌名: Clin J Am Soc Nephrol. 2011 Mar;6(3):679-89. doi: 10.2215/CJN.06710810. Epub 2011 Mar 10.
Abstract/Text Henoch-Schönlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking. The dilemma of spontaneous recovery even in patients with severe clinical and histologic presentation and of late evolution to chronic kidney disease in patients with mild initial symptoms renders it difficult for clinicians to expose patients to treatment protocols that are not evidence-based. A better understanding of the pathophysiology of progression to chronic kidney disease in Henoch-Schönlein purpura patients could be achieved by designing prospective international multicenter studies looking at determinants of clinical and histopathological evolution as well as possible circulating and urinary markers of progression. Such studies should be supported by a database available on the web and a new histologic classification of kidney lesions. This paper reports clinical, pathologic, and experimental data to be used for this strategy and to assist clinicians and clinical trial designers to reach therapeutic decisions.

PMID 21393485  Clin J Am Soc Nephrol. 2011 Mar;6(3):679-89. doi: 10.22・・・
著者: P Niaudet, R Habib
雑誌名: Pediatr Nephrol. 1998 Apr;12(3):238-43.
Abstract/Text Between 1980 and 1994, 38 children with severe forms of Schönlein-Henoch purpura glomerulonephritis were entered into a prospective study to evaluate methylprednisolone pulse therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes. The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome and/or had 50% or more crescentic glomeruli. Initial renal biopsies were obtained from all patients and revealed diffuse proliferative endocapillary glomerulonephritis in 2, focal and segmental glomerulonephritis in 4, and endo- and extracapillary glomerulonephritis in 32, 21 of whom had 50% or more glomeruli with crescents. Patients were treated with intravenous pulse methylprednisolone (3 days) followed by oral prednisone (3.5 months). At the latest follow-up, 1-16 years after initiation of therapy, 27 children had clinically recovered, 3 showed minimal urinary abnormalities, 4 persistent nephropathy, and 4 had progressed to end-stage renal failure. Sequential renal biopsies were obtained from 30 patients, 7-25 months after initiation of therapy. The clinical outcome correlated well with of the activity (hypercellularity, cellular and fibrocellular crescents, and interstitial edema with mononuclear cell infiltrates) and the chronicity (fibrous crescents, glomerular sclerosis, tubular atrophy, and interstitial fibrosis) indexes of post-therapy biopsies. Of particular interest were the post-therapy biopsies of the 18 patients who clinically recovered. They showed a significant decrease of the activity index from 5.1+/-1.1 to 0.4+/-0.8 with a decrease or even a disappearance of IgA deposits, while the chronicity index remained low (0.4+/-0.8 compared with 1.4+/-1). Although uncontrolled, our study suggests that methylprednisolone pulse therapy is effective in those patients at risk of progression of their nephropathy, especially if started early during the course of the disease before the crescents become fibrous.

PMID 9630046  Pediatr Nephrol. 1998 Apr;12(3):238-43.
著者: Penina Tarshish, Jay Bernstein, Chester M Edelmann
雑誌名: Pediatr Nephrol. 2004 Jan;19(1):51-6. doi: 10.1007/s00467-003-1315-x. Epub 2003 Nov 22.
Abstract/Text Nephritis in Henoch-Schönlein purpura (HSP) is the primary cause of morbidity and mortality. Although many therapeutic regimens have been reported to be effective, no therapy has been shown in a controlled trial to be beneficial. Fifty-six patients with histopathologically severe HSP nephritis were randomized to receive supportive therapy with or without cyclophosphamide, 90 mg/m(2)/day for 42 days. Patients were classified according to status at final follow-up: Fully Recovered 48.2%, Persistent Abnormalities 39.3%, or ESRD/Death 12.5%. There were no differences in onset data or outcome between the two trial groups or in outcome between trial and 23 non-trial patients followed concurrently. Therefore, data from trial and non-trial patients were combined for further analysis. There was no correlation between outcome and age, blood pressure, serum total protein, or serum albumin. Although rates of proteinuria did not correlate with outcome, all those with progression to ESRD had nephrotic levels of proteinuria at onset. Only five of 28 patients with nephrotic levels of proteinuria and severe onset histopathology recovered fully. No patient with crescents in 50% or more of glomeruli went on to full recovery. Recurrence of non-renal symptoms did not correlate with outcome. Nephrotic syndrome, decreased GFR, and more severe histopathology at onset, as well as persistence of urinary abnormalities for several years, are ominous signs.

PMID 14634864  Pediatr Nephrol. 2004 Jan;19(1):51-6. doi: 10.1007/s004・・・
著者: A Oner, K Tinaztepe, O Erdogan
雑誌名: Pediatr Nephrol. 1995 Feb;9(1):6-10.
Abstract/Text Twelve patients with Henoch-Schönlein purpura, aged 6-14 years (mean 10.3 years), presenting with rapidly progressive glomerulonephritis (RPGN) were investigated prospectively. Analysis of the initial clinical features revealed: oedema (8 patients), hypertension (7 patients), gross haematuria (11 patients), oliguria (5 patients) and a decreased glomerular filtration rate (GFR) (< 40 ml/min per 1.73 m2, 8 patients). Renal biopsies were available in 9 patients and revealed focal necrotising and a fibroepithelial type of crescentic glomerulonephritis (with 60%-90% crescent formation). The remaining 3 patients fulfilled the clinical criteria of RPGN. Two patients who were in the acute stage required peritoneal dialysis for a period of 2 weeks. The treatment protocol in all patients consisted of intravenous pulse methylprednisolone (3 days), oral cyclophosphamide (2 months), oral dipyridamole (6 months) and oral prednisolone (3 months). At the end of triple therapy, GFR returned to normal in all but 1 patient. During a follow-up period of 9-39 months, 7 patients achieved complete remission, while 4 patients showed partial remission, 3 of whom had persistent proteinuria and haematuria and 1 microscopic haematuria only. One patient had persistent nephropathy with decreased GFR and macroscopic haematuria and nephrotic-range proteinuria. His renal biopsy, performed 30 months after the onset of the disease, showed chronic diffuse sclerosing glomerulonephritis and intratubular severe IgA deposition. Although our patient group was small, this type of intensive treatment appears to be effective; further studies are needed.

PMID 7742225  Pediatr Nephrol. 1995 Feb;9(1):6-10.
著者: K Iijima, S Ito-Kariya, H Nakamura, N Yoshikawa
雑誌名: Pediatr Nephrol. 1998 Apr;12(3):244-8.
Abstract/Text From 1980 through 1992, 14 children with Henoch-Schönlein nephritis (HSN) showing severe glomerular changes (grade IV or V) were given a multiple combined therapy with prednisolone, cyclophosphamide, heparin/warfarin, and dipyridamole, and were followed for 7.5+/-0.9 years. The period between the onset of nephritis and the start of therapy was 0.8+/-0.4 years. Ten patients underwent follow-up biopsy after therapy. The percentage of glomeruli having crescents/segmental lesions was significantly reduced after therapy (70%+/-5% vs. 42%+/-7%, P <0.01), due mainly to the resolution of crescents (51%+/-8% vs. 13%+/-5%, P <0.01). Thus, histological grade was significantly improved (5 grade IV and 5 grade V vs. 7 grade III and 3 grade IV, P <0.01). After an average follow-up period of 7.5 years, 9 patients showed normal urine and renal function, 4 showed minor urinary abnormalities, and 1 heavy proteinuria. No patient developed chronic renal insufficiency. These findings suggest that the multiple combined therapy could be effective. for histologically severe HSN, although a prospective controlled study should be performed.

PMID 9630047  Pediatr Nephrol. 1998 Apr;12(3):244-8.
著者: Yukihiko Kawasaki, Junzo Suzuki, Hitoshi Suzuki
雑誌名: Nephrol Dial Transplant. 2004 Apr;19(4):858-64. doi: 10.1093/ndt/gfg617.
Abstract/Text BACKGROUND: There have been few controlled studies of combined therapy with multiple drugs, including immunosuppressives, for severe Henoch-Schoenlein nephritis (HSPN). We evaluated the efficacy of methylprednisolone and urokinase pulse therapy combined with cyclophosphamide for patients with HSPN of at least grade IVb.
METHODS: We studied 37 patients who had been diagnosed with HSPN of at least grade IVb. Of them, 20 (Group A) were treated with methylprednisolone and urokinase pulse therapy, and 17 (Group B) were treated with methylprednisolone and urokinase pulse therapy combined with cyclophophamide. We analysed the clinical features, laboratory and pathological findings of the two groups retrospectively.
RESULTS: After 6 months of treatment, mean urinary protein excretion in Group B had significantly decreased compared with Group A, and the activity index of both groups at the second biopsy was lower than that at the first. Furthermore, at the second biopsy, the chronicity index of Group B was lower than that of Group A. Four patients of Group A but none of Group B had persistent nephropathy (P<0.05).
CONCLUSIONS: Our study suggests that methylprednisolone and urokinase pulse therapy combined with cyclophosphamide is useful for patients with severe HSPN.

PMID 15031341  Nephrol Dial Transplant. 2004 Apr;19(4):858-64. doi: 10・・・
著者: R H Kauffmann, D A Houwert
雑誌名: Clin Nephrol. 1981 Sep;16(3):155-60.
Abstract/Text Two adult patients with Henoch-Schoenlein purpura and rapidly progressive glomerulonephritis were treated with plasmapheresis. One patient also received cyclophosphamide. Both patients recovered their renal function. Before plasmapheresis circulating IgA immune complexes were demonstrated in both patients by two assays with specificity for IgA. The level of IgA immune complexes decreased after each plasma exchange. IgA immune complexes disappeared in the patient who was treated with cyclophosphamide but remained present in the other patient. Plasma exchange may be a useful form of therapy for patients with Henoch-Schoenlein purpura and progressive renal failure. Measurement of circulating IgA immune complexes may provide insight into the in vivo effect of plasmapheresis.

PMID 7296974  Clin Nephrol. 1981 Sep;16(3):155-60.
著者: M Hattori, K Ito, T Konomoto, H Kawaguchi, T Yoshioka, M Khono
雑誌名: Am J Kidney Dis. 1999 Mar;33(3):427-33.
Abstract/Text To clarify the therapeutic role of plasmapheresis (PP) for patients with Henoch-Schönlein purpura (HSP) nephritis, the clinical courses of nine children with a rapidly progressive type of HSP nephritis, who were treated with PP as the sole therapy, were retrospectively evaluated. All patients had nephrotic-range proteinuria (4.9 +/- 2.5 g/m2/d, mean +/- SD) and decreased glomerular filtration rate (GFR) (46.5 +/- 9.5 mL/min/1.73 m2) at the time of the initiation of PP. Biopsy specimens taken before PP showed large crescents involving more than 50% of the glomerular circumference in 56.8 +/- 6.9% of the glomeruli examined. The mean interval between disease onset and initiation of PP was 39.1 +/- 22.1 days. The PP regimen consisted of thrice-weekly treatment for 2 weeks, then weekly treatment for 6 weeks. No patients received any steroids or cytotoxic drugs, except for the use of steroids to manage severe abdominal pain. All patients responded promptly to PP with improvement in renal function, reduction of proteinuria, and subsidence of purpuric rash and abdominal pain. Six of nine patients showed further improvements without any other treatments; four had complete recovery, and two had only microscopic hematuria at the latest observation (follow-up period, 9.6 +/- 4.3 years). The remaining three patients showed a rebound increase of proteinuria after completion of PP; two of whom progressed to end-stage renal failure at 14.1 years and 1.8 years after disease onset. Because all patients had the most severe forms of nephritis, reported to carry a grave prognosis, this study suggests that PP as the sole therapy is effective in improving the prognosis of patients with rapidly progressive HSP nephritis, particularly if instituted early in the course of the disease. The role of PP in treating HSP nephritis deserves to be assessed further in larger randomized controlled trials.

PMID 10070905  Am J Kidney Dis. 1999 Mar;33(3):427-33.
著者: Evangéline Pillebout, Corinne Alberti, Loic Guillevin, Amel Ouslimani, Eric Thervet, CESAR study group
雑誌名: Kidney Int. 2010 Sep;78(5):495-502. doi: 10.1038/ki.2010.150. Epub 2010 May 26.
Abstract/Text Henoch Schönlein Purpura (HSP) is a common disease in children, usually associated with a good prognosis. In adults there are no prospective studies concerning its prognosis or treatment, especially in cases of severe visceral involvement. Here we compared steroid therapy without or with cyclophosphamide co-treatment in adults with severe HSP in a 12-month, multi-center, prospective, open-label trial that treated 54 adults with biopsy-proven HSP including proliferative glomerulonephritis and severe visceral manifestations. All received steroids; however, 25 were randomized to also receive cyclophosphamide. The primary endpoint that occurred in three patients in each group was complete disease remission defined as zero on the Birmingham Vasculitis Activity Score with no persistent or new clinical and/or biological vasculitis at 6 months. No patient had active visceral involvement. The secondary endpoints were renal outcome, deaths, and adverse events at 12 months. Renal function, proteinuria, safety data, incidence of diabetes, and severe infections were similar between the two groups. At the last follow-up, renal function remained stable. The small population size of our study does not permit definitive conclusions; however, we suggest that treatment of adults with severe HSP by adding cyclophosphamide provides no benefit compared with steroids alone.

PMID 20505654  Kidney Int. 2010 Sep;78(5):495-502. doi: 10.1038/ki.201・・・
著者: Outi Jauhola, Jaana Ronkainen, Helena Autio-Harmainen, Olli Koskimies, Marja Ala-Houhala, Pekka Arikoski, Tuula Hölttä, Timo Jahnukainen, Jukka Rajantie, Timo Ormälä, Matti Nuutinen
雑誌名: Pediatr Nephrol. 2011 Dec;26(12):2159-66. doi: 10.1007/s00467-011-1919-5. Epub 2011 May 28.
Abstract/Text Knowledge about how to treat severe Henoch-Schönlein nephritis (HSN) is scarce. The aim of our study is to compare cyclosporine A (CyA) and methylprednisolone pulses (MP) in the treatment of severe HSN. Out of 24 pediatric HSN patients with nephrotic-range proteinuria or crescentic HSN in kidney biopsy, seven were randomized to receive CyA for 12 months at an initial dose of 5 mg/kg and eight to receive 3 MP pulses of 30 mg/kg followed by prednisone for 4 months. The other nine patients received identical treatment without randomization. Kidney biopsies were performed at inclusion and after 2 years. The primary outcomes were the duration of proteinuria and hematuria, estimated glomerular filtration rate, and renal biopsy histology. All the 11 CyA-treated patients achieved resolution of nephrotic-range proteinuria within 3 months, while the MP-group response was slower, and in 6/13 was not achieved with the initial treatment. Additional immunosuppressive treatment was needed in none of the CyA-treated patients but in six patients treated with MP (difference in proportion 46%, p = 0.008). The 2-year control biopsies were similarly improved in both groups. After mean 6.1 years (2.2-10.4 years), 16 patients (eight CyA, eight MP) had no renal symptoms and six (three CyA, three MP) had persistent nephropathy but normal renal function. One MP-treated patient had reduced renal function and another had developed ESRD and received a renal transplant. CyA gave a 100% resolution of nephrotic-range proteinuria and a 100% renal survival rate without additional therapy after a mean follow-up of 6 years. Treatment of HSN with CyA is efficacious, safe and not inferior to MP.

PMID 21626222  Pediatr Nephrol. 2011 Dec;26(12):2159-66. doi: 10.1007/・・・
著者: Jee Min Park, Sung Chul Won, Jae Il Shin, Hyunee Yim, Ki Soo Pai
雑誌名: Pediatr Nephrol. 2011 Mar;26(3):411-7. doi: 10.1007/s00467-010-1723-7. Epub 2010 Dec 24.
Abstract/Text To evaluate the therapeutic role of cyclosporin A (CyA) for the treatment of Henoch-Schönlein nephritis (HSN), 29 patients (18 boys, 11 girls) with nephrotic-range proteinuria were analyzed retrospectively. Mean age was 8.6 years (range 2.0-15.5 years) at diagnosis of Henoch-Schönlein purpura (HSP). All patients had developed the nephrotic-range proteinuria at a mean interval of 4.4 months (range 0-50.7 months) after the diagnosis of HSP. Mean duration of CyA treatment was 12.3 months (range 2.6-55.0 months). Mean follow-up times were 3.7 years (range 1.2-12.9 years) from the beginning of the CyA treatment. Steroids were tapered off and stopped gradually after initiation of CyA. All patients responded to the CyA treatment within a mean of 1.8 months (range 1 week to 3.5 months). Twenty-three patients achieved stable remission with mean follow-up duration of 3.2 years and 6 patients seemed to become CyA-dependent, since they developed proteinuria when the treatment was stopped. Renal function was preserved in all patients but one who developed end-stage renal disease after poor compliance with CyA. We concluded that CyA treatment for HSN showing nephrotic-range proteinuria is very effective and a safe method, although some patients become CyA-dependent.

PMID 21184240  Pediatr Nephrol. 2011 Mar;26(3):411-7. doi: 10.1007/s00・・・
著者: F T Saulsbury
雑誌名: Medicine (Baltimore). 1999 Nov;78(6):395-409.
Abstract/Text Henoch-Schönlein purpura (HSP) is an acute leukocytoclastic vasculitis that primarily affects children. In the current report, the author presents the clinical features of 100 children with HSP and reviews the literature, placing particular emphasis on new information concerning the etiology, immunopathogenesis, and treatment of HSP. The dominant clinical features of HSP are cutaneous purpura (100%), arthritis (82%), abdominal pain (63%), gastrointestinal bleeding (33%), and nephritis (40%). The etiology of HSP remains unknown, but it is clear that IgA plays a critical role in the immunopathogenesis of HSP, as evidenced by increased serum IgA concentrations, IgA-containing circulating immune complexes, and IgA deposition in vessel walls and renal mesangium. There are 2 subclasses of IgA, but HSP is associated with abnormalities involving IgA1 exclusively, and not IgA2. This finding may be a consequence of abnormal glycosylation of O-linked oligosaccharides unique to the hinge region of IgA1 molecules. Although several lines of evidence suggest a genetic susceptibility to HSP, the fundamental basis for the abnormalities involving IgA remain unclear. In general, HSP is an acute, self-limited illness, but one-third of patients will have 1 or more recurrences of symptoms. Corticosteroid therapy may hasten the resolution of arthritis and abdominal pain, but does not prevent recurrences. To date, no form of therapy has been shown to shorten appreciably the duration of HSP. The long-term prognosis of HSP is directly dependent on the severity of renal involvement. Corticosteroids in usual doses have no effect on established nephritis. Evidence is emerging that treatment with high-dose intravenous pulse methylprednisolone coupled with azathioprine or cyclophosphamide may be beneficial in patients with severe nephritis.

PMID 10575422  Medicine (Baltimore). 1999 Nov;78(6):395-409.
著者: Sandra Trapani, Annalisa Micheli, Francesca Grisolia, Massimo Resti, Elena Chiappini, Fernanda Falcini, Maurizio De Martino
雑誌名: Semin Arthritis Rheum. 2005 Dec;35(3):143-53. doi: 10.1016/j.semarthrit.2005.08.007.
Abstract/Text OBJECTIVE: To examine epidemiological, clinical, and outcome in Italian children affected with Henoch Schönlein purpura (HSP).
METHODS: Retrospective study of children discharged with a diagnosis of HSP from the Meyer Children's Hospital, between 1998 and 2002. Epidemiological, clinical, laboratory data, treatment, and outcome were collected by reviewing medical charts. One year after data collection, the children's parents were interviewed by telephone about the outcome.
RESULTS: 150 children entered the study: M:F=1.8:1; mean age 6.1+/-2.7 years. At onset, purpura was present in all cases, arthritis/arthralgias in 74%, abdominal involvement in 51%, scrotal edema in 13%, renal involvement in 54%, severe nephropathy in 7%, acute renal insufficiency in 2%, and intussusception in 0.6%. Purpura was the presenting symptom in 74%, arthritis in 15%, and abdominal pain in 12%. The most frequent laboratory abnormalities were high-erythrocyte sedimentation rate (ESR) (57%), hyper-IgA (37%), and proteinuria (42%). All patients recovered within 2 months. Recurrences, verified in 35%, were correlated with high ESR values and corticosteroid (CS) treatment, independently from other variables. After a mean 2.5-years follow-up, 2 patients had hematuria with normal renal function.
CONCLUSION: Epidemiological and clinical findings in our cohort are similar to those in the literature, even though the mean disease duration was shorter than previously reported. Relapses occurred significantly more frequently in children treated with CS. This finding supports the recommendation to limit the use of steroids to a carefully selected group of HSP children. The prognosis was excellent; although severe nephropathy was found in a small percentage of the children, at follow-up all had normal renal function. Thus, our study confirms the benignity of HSP in Italian children, especially regarding renal outcome.

PMID 16325655  Semin Arthritis Rheum. 2005 Dec;35(3):143-53. doi: 10.1・・・
著者: M Levy, M Broyer, A Arsan, D Levy-Bentolila, R Habib
雑誌名: Adv Nephrol Necker Hosp. 1976;6:183-228.
Abstract/Text
PMID 139081  Adv Nephrol Necker Hosp. 1976;6:183-228.
著者: H A Austin, J E Balow
雑誌名: Am J Kidney Dis. 1983 Mar;2(5):512-20.
Abstract/Text Henoch-Schönlein purpura is an intriguing entity of uncertain etiology associated with circulating IaA immune complexes, enhanced spontaneous immunoglobulin production, and an apparent imbalance in T cell regulatory functions. The associated glomerulonephritis is highly variable in nature and may profoundly influence clinical outcome. Prognostic features are reviewed in an effort to identify high-risk patients. Age, non-renal manifestations, non-selectivity of proteinuria, and evidence of preceding streptococcal infection are relatively weak predictors of end-stage renal disease. The presenting clinical and histologic expressions of nephritis are more useful prognosticators, but neither are completely reliable. Patients with greater than 50% crescents, and those with nephrotic syndrome complicated by various combinations of hypertension, azotemia, oliguria, and/or hypoproteinemia, are at increased risk of renal failure and might benefit from therapeutic interventions. Experience with various modalities is reviewed. Faced with uncontrolled clinical data, it is unclear whether immunosuppressive agents or plasmapheresis offer a therapeutic advantage over oral corticosteroids alone.

PMID 6338708  Am J Kidney Dis. 1983 Mar;2(5):512-20.
著者: S Shrestha, N Sumingan, J Tan, H Alhous, H Althous, L McWilliam, F Ballardie
雑誌名: QJM. 2006 Apr;99(4):253-65. doi: 10.1093/qjmed/hcl034.
Abstract/Text BACKGROUND: Henoch Schönlein purpura with nephritis (HSN) in adults may cause severe organ injury, but its rarity has contributed to a lack of data.
AIM: To evaluate clinical outcomes and risk factors in adult HSN patients.
DESIGN: Retrospective analysis.
METHODS: Thirty-seven patients with adult HSN attending the Regional Vasculitis Clinic between 1974 and 2004 were assessed. For inclusion, a renal biopsy showing predominant mesangial IgA immune deposits was required, plus at least two of: purpuric rash, arthralgia, abdominal pain.
RESULTS: Ten patients (27%) progressed to end-stage renal failure (ESRF). Renal failure rates were highest in the first decade, with survival rate 72% at 5 years, 68% at 10 years and 46% at final review. Risk factors for ESRF were: proteinuria > or =1 g/day during follow-up (RR 83.8, p = 0.0006); hypertension at presentation (RR = 53.3, p = 0.0045) and during follow-up (RR = 5.9, p = 0.05); renal impairment at presentation (RR 8.0, p = 0.0015); age <30 years (RR 7.6, p = 0.02); and male sex (RR = 6.0, p = 0.05). Biopsies frequently showed crescents, mostly affecting <50% of glomeruli; their presence predicted ESRF, as did interstitial fibrosis and tubular atrophy. Renal remission, in contrast, was also high (43%). Cytotoxics were used in 32%, with no clear effect on outcome. Relapses affecting the classical extra-renal systems were common, but were not associated with declines in renal function. A high proportion of patients (41%) also suffered vasculitic organ injuries outside the classical systems.
DISCUSSION: HSN in adults is a serious relapsing disease, causing renal failure as frequently as in small-vessel ANCA-positive vasculitides. Prognosis and risks differed in this series from those in other countries, including a higher risk of ESRF than in previous series. Distinct groups developed either ESRF, or remitted. The absence of clear benefit suggests that corticosteroids should be reserved for patients with serious disease, and that cytotoxics may not be merited for those at high risk of renal failure.

PMID 16565522  QJM. 2006 Apr;99(4):253-65. doi: 10.1093/qjmed/hcl034.
著者: Q Meulders, Y Pirson, J P Cosyns, J P Squifflet, C van Ypersele de Strihou
雑誌名: Transplantation. 1994 Dec 15;58(11):1179-86.
Abstract/Text The frequency and the risk factors for clinical recurrence of Henoch-Schönlein nephritis following renal transplantation (TP) remain largely unknown. We report on 14 transplants performed at our center in 10 patients, detail the evolution of 2 of them with clinical recurrence, and review 64 other transplants reported in the literature. In our series, all patients are currently alive. Seven grafts are well-functioning 22-295 (mean, 97) months after TP without any sign of clinical recurrence. Five grafts were lost from rejection. Clinical recurrence occurred in 2 patients who were on cyclosporine/azathioprine/prednisone therapy. Pooling our series with that of Hasegawa et al., the actuarial risk for renal recurrence and for graft loss due to recurrence was 35 and 11% at 5 years after TP, respectively. In our series, duration of original disease was 2 and 28 months in the 2 patients with recurrence versus 31-144 months in the others without recurrence. In the literature, this duration was < or = 36 months in all 7 patients with recurrence. Recurrence occurred despite a > 12-month delay between disappearance of purpura and TP in our 2 patients and in 3 of 6 previously reported recurrences. We conclude that Henoch-Schönlein purpura nephritis frequently recurs after TP. Recurrence (1) seems to be associated with a shorter duration of the original disease, (2) can occur despite a delay of more than 1 year (as commonly advised) between disappearance of purpura and TP, and (3) is not prevented by a triple immunosuppressive regimen that includes cyclosporine.

PMID 7992359  Transplantation. 1994 Dec 15;58(11):1179-86.
著者: Nada Kanaan, Georges Mourad, Eric Thervet, Patrick Peeters, Maryvonne Hourmant, Yves Vanrenterghem, Martine De Meyer, Michel Mourad, Céline Maréchal, Eric Goffin, Yves Pirson
雑誌名: Clin J Am Soc Nephrol. 2011 Jul;6(7):1768-72. doi: 10.2215/CJN.00520111.
Abstract/Text BACKGROUND AND OBJECTIVES: The actuarial risk at 5 years for clinical recurrence of Henoch-Schönlein purpura nephritis (HSPN) and graft loss caused by recurrence of -HSPN after renal transplantation was reported in 1994 to be as high as 35% and 11%, respectively. The aim of this study is to re-evaluate, in a large cohort of patients with a long-term follow-up, whether these rates have changed.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Patients from six transplant centers in Belgium and France with strict diagnostic criteria of HSPN and a potential post transplant follow-up of ≥3 years were included.
RESULTS: Forty-three patients were included. Patient survival is excellent: 98%, 95%, and 95% at 5, 10, and 15 years, respectively. Overall graft survival rates were 84%, 66%, and 56% at 5, 10, and 15 years, respectively. Clinical recurrence in a first kidney transplant occurred in five patients. Three patients lost their first graft due to HSPN recurrence 19 to 96 months after transplantation, two of whom had systemic signs of the illness. Actuarial risk for clinical recurrence in a first graft is 2.5% and 11.5% at 5 and 10 years, respectively. Actuarial risk for graft loss caused by recurrence in a first graft is 2.5% and 7.5% at 5 and 10 years, respectively. Severity of the disease at presentation and type of immunosuppression after transplantation did not affect recurrence.
CONCLUSIONS: We found that recurrence rates of HSPN after transplantation are lower than previously reported. The actuarial risk of graft loss from recurrence in a first graft is 7.5% at 10 years.

PMID 21734091  Clin J Am Soc Nephrol. 2011 Jul;6(7):1768-72. doi: 10.2・・・
著者: Joyce P Samuel, Cynthia S Bell, Donald A Molony, Michael C Braun
雑誌名: Clin J Am Soc Nephrol. 2011 Aug;6(8):2034-40. doi: 10.2215/CJN.01410211. Epub 2011 Jun 23.
Abstract/Text BACKGROUND AND OBJECTIVES: Although Henoch-Schönlein purpura (HSP) is the most common form of renal vasculitis in childhood, progression to ESRD is rare, and there are few data on outcomes of renal transplantation in patients with HSP.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This is a matched retrospective cohort study of renal allografts using the United Network of Organ Sharing database (1987 to 2005). Of the 189,211 primary renal allografts, there were 339 with a diagnosis of HSP. The primary end point was allograft survival.
RESULTS: Compared with the remainder of the database, the HSP population was younger (25 years versus 46 years), and had a higher proportion of women (47% versus 40%), live donors (50% versus 35%), and Caucasians (77% versus 60%). Controlling for age, gender, donor source, ethnicity, and year of transplantation, death-censored graft survival for patients with HSP was 80.0% at 5 years and 58.8% at 10 years compared with 79.0% at 5 years and 55.4% at 10 years in the non-HSP population. Among patients with reported causes of graft loss, failure from recurrent disease occurred in 13.6% of patients with HSP, compared with 6.6% in the non-HSP population. When analyzing allograft survival in recipients with HSP compared with those with IgA nephropathy, there was no difference in 10-year allograft survival (58.4% and 59.3%, respectively).
CONCLUSIONS: These data indicate that although there is an increased risk of graft failure attributable to recurrent disease in patients with HSP, a diagnosis of HSP has little effect on overall renal allograft survival.

PMID 21700827  Clin J Am Soc Nephrol. 2011 Aug;6(8):2034-40. doi: 10.2・・・

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