今日の臨床サポート

過敏性血管炎

著者: 金子駿太 JCHO 東京山手メディカルセンター

監修: 金子礼志 国立国際医療研究センター 膠原病科

著者校正済:2021/12/15
現在監修レビュー中
参考ガイドライン:
  1. 日本皮膚科学会:血管炎・血管障害診療ガイドライン2016年改訂版
  1. 日本循環器学会ほか:血管炎症群の診療ガイドライン2017年改訂版
患者向け説明資料

概要・推奨   

  1. 成人で下腿中心の紫斑をみたときには、血小板数と凝固(PT、APTT)測定を行い、正常な場合には皮膚血管炎を疑う。血管炎の証明のために皮膚生検を行うことが推奨される。同時に他臓器病変の有無を確かめる(推奨度2)
  1. 20歳未満の場合に、下腿中心の紫斑で血小板減少や凝固異常がなく、皮膚の血管炎が疑われる場合は、ほとんどがHenoch-Schonlein紫斑病(IgA血管炎)であり、皮膚生検を行う必要はない(推奨度2)
  1. 皮膚生検にて皮膚白血球破砕性血管炎を認めた場合には、過敏性血管炎を考えて薬剤使用歴、先行感染の有無を確認する(推奨度2)
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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。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
  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。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。閲覧
  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。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。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
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:
金子駿太 : 未申告[2021年]
監修:金子礼志 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期的なレビューを行った(変更なし)。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 過敏性血管炎とは、もともと薬剤やウイルス、細菌などに対して免疫系が過剰反応を起こして、主に皮膚の血管に炎症が起こる疾患として定義された。
  1. 皮膚生検にて、皮膚真皮浅層の後毛細血管静脈を病変の首座とし、血管壁や血管周囲に浸潤した好中球に由来した核破砕を特徴とした白血球破砕性血管炎(leukocytoclastic vasculitis)を認める。
  1. 病態にⅢ型アレルギー(免疫複合体)が関与しているとされており、間接蛍光抗体法にて免疫グロブリンや補体の沈着がみられるが、免疫複合体のうち、免疫グロブリンは血管壁からの乖離、炎症細胞による貪食によって48時間後にほぼ消失するため皮膚血管炎の生検は皮疹出現後24~48時間以内の施行が望ましい。
  1. 近年、血管炎のさまざまな原因が明らかになるに伴い、血管炎は病因により分類されるようになったため、過敏性血管炎の名称が使われることは少ない。血管炎の国際分類を定めた会議(Chapel Hill Consensus Conference:CHCC)では1994年に過敏性血管炎は除かれた。最新のCHCC2012では過敏性血管炎は以下に分類される。誘因の推定される続発性血管炎として原因の明らかなものは、薬剤関連免疫複合体性血管炎/薬剤関連ANCA関連血管炎/C型肝炎関連クリオグロブリン性血管炎/B型肝炎ウイルス関連血管炎/HIV関連血管炎/癌関連血管炎、原因が明らかでなく皮膚症状が主体の血管炎は皮膚白血球破砕性血管炎、IgA沈着を認めて皮膚以外にも消化管、腎炎(紫斑病性腎炎)をしばしば生じる血管炎はIgA血管炎/Henoch-Schonlein紫斑病である。
 
Chapel Hill Consensus Conference 2012(CHCC2012)の分類と過敏性血管炎

  1. CHCC2012では、罹患血管径による分類を基本として、病因の明らかなものは別に分類している。過敏性血管炎は薬剤、感染等の誘因が推定される血管炎の中で皮膚症状が主体であるものと、誘因の明らかでない皮膚白血球破砕性血管炎、IgA血管炎が該当する。
  1. 誘因が推定される血管炎はC型肝炎ウイルス関連クリオグロブリン血症性血管炎、C型肝炎以外の感染症関連クリオグロブリン血症性血管炎、B型肝炎ウイルス関連血管炎、薬剤関連免疫複合体性血管炎、薬剤関連ANCA 関連血管炎、癌関連血管炎、HIV関連血管炎等の誘因が含まれる。
  1. IgA血管炎は薬剤や感染などの関連が強いため、過敏性血管炎の特殊型といえる。

出典

img1:  2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
 
 Arthritis Rheum. 2013 Jan;65(1):1-11. do・・・
 
過敏性血管炎に該当する疾患のまとめ

Chapel Hill Consensus Conference(CHCC2012)の分類では、過敏性血管炎の名称は使われておらず、原因により細分化されている。
過敏性血管炎は、皮膚以外の全身症状は少ないとされているが、これら原因ごとの疾患分類では、全身症状を伴う場合がある。共通していることは、皮疹は紫斑が多いこと、病理組織は皮膚白血球破砕性血管炎であること、軽症例では原因の除去や対症療法が主体で免疫抑制療法は必要ないこと。頻度は少ないが重症例では免疫抑制剤併用や血漿交換など集学的な治療が必要になることである。
 
参考文献:
日本皮膚科学血管炎・血管障害診療ガイドライン改訂版作成委員会: 薬剤関連血管炎. 血管炎・血管障害診療ガイドライン2016年改訂版.日皮会誌:127(3),299-415,2017.
Bahrami S, Malone JC, Webb KG. Tissue eosinophilia as an indicator of drug-induced cutaneous small-vessel vasculitis. Arch Dermatol. 2006 Feb;142(2):155-61. PMID: 16490843
Loricera J, Calvo-Río V, Ortiz-Sanjuán F. The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features. Medicine (Baltimore). 2013 Nov;92(6):331-43. PMID: 24145696

出典

img1:  狩野俊和先生提供
 
 
 
  1. 全身性血管炎の皮膚症状として白血球破砕性血管炎がみられることもある。ANCA関連血管炎(顕微鏡的多発血管炎、多発血管炎性肉芽腫症、好酸球性多発血管炎性肉芽腫症)、クリオグロブリン血症性血管炎(特発性、一部は続発性)、蕁麻疹様血管炎、ループス血管炎、リウマチ性血管炎の急性期も同所見をみるため、これらの疾患の除外が必要である。
  1. IgA血管炎は過敏性血管炎の特殊型で、腎や腸管にも血管炎による血尿や腹痛、下血が生じる。糸球体腎炎合併頻度は成人で50~80%、小児で20~50%とされる。男性に多く、季節的には冬季に多い傾向がある。
  1. IgA血管炎の一部は溶連菌感染との関連があり、上気道感染の1~2週後に起きることが多い。小児に多い疾患であるが、成人にも生じる。治療は原因の除去が大事なので、関与が疑われる場合は薬剤中止、細菌感染の場合は抗菌薬投与を行う。
  1. 皮膚血管炎の20~30%で薬剤が関与するとの意見があり、原因薬剤の早期中止により重症化の防止が可能で、薬剤中止のみで寛解する症例もある。過度の免疫抑制療法を避けるため、薬剤関連血管炎は常に考慮する必要がある。ANCAの関与する薬剤関連ANCA関連血管炎と関与しない薬剤関連免疫複合体性血管炎がある。
  1. クリオグロブリンが陽性の血管炎は、多くがHCV陽性であり、C型肝炎関連クリオグロブリン血症性血管炎と呼ばれる。紫斑、関節痛、多発神経障害、腎障害を認める。アルゴリズムアルゴリズム
  1. HBV感染に伴う血管炎は結節性多発動脈炎が多いとされるが、わが国での頻度は少ない。他に皮膚白血球破砕性血管炎や蕁麻疹様血管炎、クリオグロブリン血症性血管炎の報告もある。
  1. 血液悪性腫瘍などに伴って皮膚血管炎が生じることがある。皮膚症状の多くがpalpable purpuraであり、難治性の場合には合併を考慮する必要がある。
  1. 原因が明らかでないものは皮膚白血球破砕性血管炎である。
問診・診察のポイント  
  1. 皮膚以外の全身症状を確認する。

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文献 

著者: R Blanco, V M Martínez-Taboada, V Rodríguez-Valverde, M García-Fuentes
雑誌名: Medicine (Baltimore). 1998 Nov;77(6):403-18.
Abstract/Text Cutaneous vasculitis (CV), a condition characterized by palpable purpura and nonspecific histopathologic findings, presents a diagnostic and therapeutic challenge because it may be a primary disorder or it may be a cutaneous manifestation of another entity, such as systemic necrotizing vasculitis, connective tissue disease, systemic bacterial infection, or malignancy. We studied 303 unselected patients (172 adults and 131 children) with CV to assess the disease associations and etiologic factors, to identify the frequency of primary and secondary CV in different age-groups, and to characterize features that help to distinguish between primary and secondary CV. Of the 131 children, 130 had primary CV: Henoch-Schönlein purpura (HSP) in 116 and hypersensitivity vasculitis (HV) in 14. In contrast, of the 172 adults, only 120 had primary CV: HSP in 39, HV in 70, and essential mixed cryoglobulinemia in 11. CV was a manifestation of systemic necrotizing vasculitis in 23 adults (polyarteritis nodosa in 17, Wegener granulomatosis in 4, and Churg-Strauss syndrome in 2). CV was secondary to other processes in 29 adults: in 20 patients CV was associated with connective tissue disease or another autoimmune or rheumatic disease, in 5 patients CV was a manifestation of severe bacterial infection, especially bacterial endocarditis (4 cases), and in the other 4 patients CV was the presenting symptom of an underlying malignancy. The patients for whom CV was a manifestation of systemic necrotizing vasculitis or secondary to a connective tissue disease, severe bacterial infection, or malignancy had clinical and laboratory data suggestive of the associated disorder. The clinical picture and outcome of primary CV in both children and adults were benign. By contrast, the prognosis of patients with CV in the context of systemic necrotizing vasculitis or secondary to other entities depended on the primary process. Given the different disease association in children and adults, we propose a simple diagnostic workup in children with CV. By contrast the diagnostic approach in adults with CV should be more cautious and the workup more extensive. The early differentiation between primary CV, secondary CV, and CV presenting as a symptom of systemic necrotizing vasculitis, especially in adults, is of paramount importance for an adequate diagnosis and appropriate treatment.

PMID 9854604  Medicine (Baltimore). 1998 Nov;77(6):403-18.
著者: M C Calviño, J Llorca, C García-Porrúa, J L Fernández-Iglesias, P Rodriguez-Ledo, M A González-Gay
雑誌名: Medicine (Baltimore). 2001 Sep;80(5):279-90.
Abstract/Text
PMID 11552081  Medicine (Baltimore). 2001 Sep;80(5):279-90.
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: Alex P Betrosian, Tom Berlet, Banwari Agarwal
雑誌名: Am J Med Sci. 2006 Dec;332(6):339-45.
Abstract/Text Sepsis-induced purpura fulminans is a rare but life-threatening disorder, characterized by hemorrhagic infarction of the skin caused by disseminated intravascular coagulation and dermal vascular thrombosis. The pathogenesis is linked to enhanced expression of the natural procoagulants and depletion of the natural anticoagulant proteins particularly protein C. Meningococcal sepsis is the most common cause, followed by pneumococcal sepsis in adults. The syndrome is associated with more than 50% mortality secondary to multiple organ dysfunction syndrome and is accompanied by long-term morbidity. Necrotic lesions usually progress to distal ischemia, and skin grafting and extremities or limb amputation are often required. Early antibiotic administration and intensive care management according to the recommendations of severe sepsis and shock is crucial for patients' survival. Adjuvant therapies against inflammatory and coagulation cascades and augmenting fibrinolysis are still controversial and need further assessment. Among them activated protein C and supplementation therapy have given promising results.

PMID 17170624  Am J Med Sci. 2006 Dec;332(6):339-45.
著者: Mark D Denton, Subba R Digumarthy, Sarah Chua, Robert B Colvin
雑誌名: N Engl J Med. 2006 Jun 29;354(26):2803-13. doi: 10.1056/NEJMcpc069012.
Abstract/Text
PMID 16807418  N Engl J Med. 2006 Jun 29;354(26):2803-13. doi: 10.1056・・・
著者: V M Martinez-Taboada, R Blanco, M Garcia-Fuentes, V Rodriguez-Valverde
雑誌名: Am J Med. 1997 Feb;102(2):186-91.
Abstract/Text PURPOSE: To evaluate the clinical features and outcome of patients with isolated hypersensitivity vasculitis (HV).
PATIENTS AND METHODS: Retrospective study of patients with cutaneous vasculitis followed up at a University Hospital from 1975 to 1994. Patients with vasculitis secondary to collagen vascular diseases, neoplasia, or major infections were excluded. Patients were classified as HV according to the differential criteria proposed by Michel et al (J Rheumatol. 1992;19:721-728).
RESULTS: Ninety-five patients were classified as HV. The mean age was 42.7 +/- 21.7 years, with similar disease frequency in both sexes. In 43 patients, the precipitating event was drug therapy, either alone or as a treatment for a coexistent infection, usually an upper respiratory tract infection. The most frequent clinical manifestation was palpable purpura followed by joint symptoms. Systemic involvement was infrequent: 7 patients had nephropathy, manifested almost exclusively by microhematuria, and 5 patients had gastrointestinal symptoms. In 54 subjects the vasculitis did not require treatment; 26 patients were treated with NSAIDs, and 14 required corticosteroids (associated to immunosuppressive agents in 2 of them). After a mean follow-up of 15.5 +/- 28.9 months (median 6), only 2 patients had slight renal impairment, whereas the remaining had a complete recovery.
CONCLUSION: Hypersensitivity vasculitis is usually a benign syndrome, often secondary to drugs or infections, or both. Its main clinical manifestations are skin and joint symptoms. The systemic involvement is scarce and its prognosis is excellent.

PMID 9217569  Am J Med. 1997 Feb;102(2):186-91.
著者: G Sais, A Vidaller, A Jucglà, F Gallardo, J Peyrí
雑誌名: Arch Dermatol. 1995 Dec;131(12):1399-402.
Abstract/Text BACKGROUND AND DESIGN: Cutaneous leukocytoclastic vasculitis is an inflammatory vascular disease with a variable course. There is no defined therapy for this entity. Contradictory data on the effect of colchicine have been reported. To determine the efficacy of colchicine in cutaneous leukocytoclastic vasculitis, 41 patients were randomly selected to receive oral colchicine, 0.5 mg twice daily, or topical emollients. Response to treatment was judged according to the reduction in the number of lesions. After 1 month, in those patients in whom a complete or no response was achieved, therapy was withdrawn; in those with a partial response, treatment was maintained for the following 2 months. At the end of 3 months, treatment was continued only in those patients in whom a relapse occurred.
RESULTS: Twenty patients in each group completed 1 month of treatment. One patient taking colchicine dropped out because of diarrhea. At the end of the first month of the study, five patients in the control group and four in the colchicine group achieved a complete response. Nine patients who had a partial response (four in the colchicine group and five in the control group) continued to receive treatment for the following 2 months. Three patients in the colchicine group suffered a relapse after discontinuing therapy but experienced remission with reinstitution of therapy. At the end of the 3 month period, 12 patients in the colchicine group and 10 patients in the control group showed no significant response. Complete response was achieved in five patients in the colchicine group and in seven in the control group. At the 1-year follow-up, 10 patients in each group had no clinical evidence of cutaneous vasculitis.
CONCLUSIONS: Colchicine had no significant therapeutic effect in this controlled study. However, the finding that relapse occurred on cessation of colchicine therapy in three complete responders suggests that colchicine can be effective in some patients, despite our negative results.

PMID 7492128  Arch Dermatol. 1995 Dec;131(12):1399-402.
著者: R Wolf, B Tüzün, Y Tüzün
雑誌名: Clin Dermatol. 2000 Jan-Feb;18(1):37-53.
Abstract/Text
PMID 10701085  Clin Dermatol. 2000 Jan-Feb;18(1):37-53.
著者: L H Calabrese, G F Duna
雑誌名: Curr Opin Rheumatol. 1996 Jan;8(1):34-40.
Abstract/Text Vasculitis resulting from drug use includes a wide variety of clinical and pathologic conditions that are, in general, empirically defined and poorly understood. Further complicating our grasp of these disorders are ambiguous terms such as hypersensitivity vasculitis, allergic vasculitis, leukocytoclastic vasculitis, serum sickness, and others, which are often used interchangeably without clear definition. The clinical picture varies widely from self-limiting to progressive and even fatal illness. These syndromes have now been reported in association with newer classes of therapeutic agents including biologic response modifiers. Vasculitis affecting the central nervous system may be related to a variety of drugs and remains one of the more important syndrome sets within the spectrum of drug-induced vasculitis. These disorders are clinically important, because removal of the offending drug often is associated with regression of the vasculitic condition.

PMID 8867537  Curr Opin Rheumatol. 1996 Jan;8(1):34-40.
著者: F G Mullick, H A McAllister, B M Wagner, J J Fenoglio
雑誌名: Hum Pathol. 1979 May;10(3):313-25.
Abstract/Text Drug related vasculitis has variously been described as necrotizing hypersensitivity or allergic angiitis or microscopic panarteritis nodosa. We reviewed tissue sections from 30 patients with validated drug hypersensitivity and vasculitis in order to precisely define this entity. No evidence of necrotizing vascular lesions or of fibrinoid associated with necrosis was found. The vascular lesions in all 30 patients involved small arteries, arterioles, capillaries, and venules. The inflammatory infiltrate consisted primarily of mononuclear cells and prominent numbers of eosinophils and was present in all three layers of the involved vessel walls. Clinically the patients developed either localized or systemic vasculitis, which could not be predicted on the basis of the associated drug. The findings of a skin rash, fever, or eosinophilia and the development of symptoms consistent with a hypersensitivity reaction while medication was being taken were all suggestive of the diagnosis of drug related vasculitis.

PMID 468217  Hum Pathol. 1979 May;10(3):313-25.
著者: C W Parker
雑誌名: Pharmacol Rev. 1982 Mar;34(1):85-104.
Abstract/Text The general features of allergic drug reactions in man have recently been reviewed by Parker (85). By definition allergic drug reactions are produced by specific immunologic processes. Allergic drug reactions must be distinguished from adverse reactions due to overdosage, normal pharmacologic action, toxic metabolite formation, idiosyncrasy, nonspecific release of pharmacologic effector molecules, or drug interactions. The clinical manifestations of drug allergy are quite protean. In addition to classical manifestations of allergy such as serum sickness, anaphylaxis, contact dermatitis or urticaria, drug allergy may produce hemolytic anemia, thrombocytopenia, granulocytopenia, hepatitis, nephritis, pneumonitis, vasculitis, or neuritis where a single organ or cell type is affected. While many drugs produce reactions with suggestive of allergy, definitive experimental evidence either for or against mechanism is usually not available. Some of these reactions may involve allergic mediators released or produced nonimmunologically through pharmacologic, osmotic, or toxic effects on cells involved in immune inflammation (mast cells, basophils, phagocytes, and lymphocytes) or through nonspecific activation of effector molecules in extracellular fluid such as the complement proteins. Drugs may also induce the formation of autoantibodies through mechanisms that are largely obscure, but may in some instances involve the direct participation of the drug as a hapten and in other instances occur indirectly through a pharmacologic or toxic action on the cells responsible for immune homeostasis.

PMID 7041144  Pharmacol Rev. 1982 Mar;34(1):85-104.
著者: Wen-Liang Chang, Yao-Hsu Yang, Li-Chieh Wang, Yu-Tsan Lin, Bor-Luen Chiang
雑誌名: Pediatr Nephrol. 2005 Sep;20(9):1269-72. doi: 10.1007/s00467-005-1903-z. Epub 2005 Jun 10.
Abstract/Text Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small vessel vasculitis of childhood. It is characterized by the symptoms including nonthrombocytopenic purpura, abdominal pain, hematuria/proteinuria, and arthargia/arthritis. We conducted a retrospective study of 261 patients diagnosed with HSP from December 1991 to December 2001. Of the 261 patients, fifty-three (20.3%) developed renal manifestations after onset of the disease. Two patients developed nephrotic syndrome. Four patients had group A beta-hemolytic streptococcal pharyngitis and subsequent depressed serum C3 concentration typical of post streptococcal glomerulonephritis. During the study period, the renal survival rate after disease onset was 100%. The prognosis of renal involvement was better than reports from other series. In this study we also found factors associated with HSP nephritis; these included older age at onset, GI bleeding, and central nervous system involvement. The long-term morbidity of HSP is predominantly attributed to renal involvement. It is thus recommended that patients with HSP nephritis are followed for longer periods of time.

PMID 15947991  Pediatr Nephrol. 2005 Sep;20(9):1269-72. doi: 10.1007/s・・・
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: 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: Tae-Sun Ha, Jin-Seok Lee
雑誌名: Acta Paediatr. 2007 Apr;96(4):552-5. doi: 10.1111/j.1651-2227.2006.00173.x. Epub 2007 Feb 14.
Abstract/Text AIM: Henoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis involving the skin, gastrointestinal tract, joint, kidneys and even scrotum.
METHODS: We retrospectively reviewed the clinical and laboratory data of 120 male patients with HSP and also evaluated the risk factors for scrotal involvement and the relation between scrotal involvement and other clinical features. Twenty-six out of 120 boys (21.7%) diagnosed with HSP had scrotal involvement.
RESULTS: Scrotal symptoms manifested as swelling in 88.5% and pain (or tenderness) in 69.2% of HSP patients with scrotal involvement. Neurologic symptoms, mainly headache and localized edema among various manifestations and high serum C3 level of laboratory profiles were more frequently observed in scrotal-involved group than in those of non-involved group. However, there was no difference in the outcomes of scrotal symptoms according to therapeutic modalities and the occurrence of scrotal involvement had no correlation with renal involvement from acute to chronic phase.
CONCLUSIONS: We found that neurologic symptoms, localized edema and high serum C3 level show a significant relation with scrotal involvement in male HSP patients. Because scrotal involvement in male HSP patients is not rare, the accurate early diagnosis of HSP is mandatory by the early notification of purpura and imaging evaluations in order to avoid unnecessary procedures.

PMID 17306010  Acta Paediatr. 2007 Apr;96(4):552-5. doi: 10.1111/j.165・・・
著者: A L Belman, C R Leicher, S L Moshé, A P Mezey
雑誌名: Pediatrics. 1985 Apr;75(4):687-92.
Abstract/Text Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 7 1/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present on initial examinations gradually resolved, but a mild left arm paresis persisted. Cutaneous, renal, and joint involvement followed initial CNS manifestations. The second patient, a 7-year-old girl, had a complex partial seizure with secondary generalization and a postictal hemiparesis seven days after presentation with classic signs of Schoenlein-Henoch purpura. Behavioral changes were noted during the acute phase of the illness. The third patient, a 13-year-old boy, developed signs of a left brachial plexopathy and transient weakness of his right leg during a complicated course of Schoenlein-Henoch purpura. Review of the world literature indicates that headaches and mental status changes are the most frequent neurologic complications of Schoenlein-Henoch purpura, followed by seizures, focal neurologic deficits, mononeuropathies, and polyradiculoneuropathies. The vasculitis of Schoenlein-Henoch purpura can involve the nervous system and may add significantly to the morbidity of the illness.

PMID 2984637  Pediatrics. 1985 Apr;75(4):687-92.
著者: A K Misra, A Biswas, S K Das, P K Gharai, T Roy
雑誌名: J Assoc Physicians India. 2004 Oct;52:833-4.
Abstract/Text Henoch-Schonlein purpura is a leucocytoclastic vasculitis commonly seen among children and young adults. Neurological complications, though rare, include focal cerebral deficit, coma, convulsion, subarachnoid hemorrhage and chorea. We are reporting a 12 years boy with Henoch-Schonlein purpura who developed a large intracerebral hematoma in right occipital lobe. He made an uneventful recovery with conservative treatment and one year follow up revealed no major neurological sequelae.

PMID 15909863  J Assoc Physicians India. 2004 Oct;52:833-4.
著者: A Bulun, R Topaloglu, A Duzova, I Saatci, N Besbas, A Bakkaloglu
雑誌名: Pediatr Nephrol. 2001 Dec;16(12):1139-41. doi: 10.1007/s004670100048.
Abstract/Text Henoch-Schönlein purpura (HSP) is a multisystemic vasculitis. Nervous system involvement is usually underestimated. Headaches, mental status changes and seizures are the most frequent neurologic symptoms. Ataxia and mononeuropathy are both very rare. We present an 11-year-old boy with HSP who suffered from ataxia during the initial presentation and peripheral neuropathy at the time of a relapse. Brainstem vasculitic involvement was shown by magnetic resonance imaging, while cranial tomography was normal. All the neurologic symptoms and signs resolved following bolus methylprednisolone administration. Ten months later he had a second course of HSP with skin and renal involvement. A percutaneous renal biopsy, which was performed due to persistent hematuria, revealed mesangial proliferation with IgA deposition. During that period the patient experienced pain and numbness in the right foot and leg; electromyography showed signs of mononeuritis multiplex involving the right posterior tibial nerve. The patient responded to steroid therapy.

PMID 11793117  Pediatr Nephrol. 2001 Dec;16(12):1139-41. doi: 10.1007/・・・
著者: K R Vats, A Vats, Y Kim, D Dassenko, A R Sinaiko
雑誌名: Pediatr Nephrol. 1999 Aug;13(6):530-4. doi: 10.1007/s004670050652.
Abstract/Text Pulmonary hemorrhage, a rare complication of Henoch-Schönlein purpura (HSP) reported primarily in adults and adolescents, is associated with significant mortality. Although it has been suggested that pulmonary hemorrhage also occurs in children with HSP, the few cases reported lack a clear differentiation from pauci-immune vasculitis. We report a prepubertal child with HSP, pulmonary hemorrhage, and immunofluorescence-documented IgA deposits on renal biopsy. Aggressive supportive management and steroid therapy led to successful recovery. A review of the current literature is presented. Because other conditions clinically mimic HSP, appropriate serological studies and a kidney biopsy to confirm the diagnosis should be performed in severely affected patients with renal disease.

PMID 10452284  Pediatr Nephrol. 1999 Aug;13(6):530-4. doi: 10.1007/s00・・・
著者: M M K Muqit, M J Gallagher, M Gavin, F Roberts, A G Jardine
雑誌名: Br J Ophthalmol. 2005 Sep;89(9):1221-2. doi: 10.1136/bjo.2004.064519.
Abstract/Text
PMID 16113386  Br J Ophthalmol. 2005 Sep;89(9):1221-2. doi: 10.1136/bj・・・
著者: H Narchi
雑誌名: Arch Dis Child. 2005 Sep;90(9):916-20. doi: 10.1136/adc.2005.074641. Epub 2005 May 4.
Abstract/Text BACKGROUND: The duration of follow up to assess the risk of long term renal impairment in Henoch-Schönlein purpura (HSP) without nephritic or nephrotic syndrome or renal failure on diagnosis remains undetermined.
AIMS: To undertake a systematic review of the literature to assess whether the risk of long term renal impairment without renal involvement on diagnosis could be estimated and to determine the time period when renal involvement is very unlikely after the diagnosis of HSP.
METHODS: Search of studies of unselected children with HSP, and available information on urinary findings, renal involvement, and long term renal function follow up. Studies of selected children with HSP nephropathy at diagnosis were excluded.
RESULTS: Twelve studies of 1133 children were reviewed. The follow up period ranged from 6 weeks to 36 years. Proteinuria and/or haematuria, which occurred in 34.2%, of which only one fifth were in association with nephritic or nephrotic syndrome, developed in 85% of cases within 4 weeks of the diagnosis of HSP, in 91% within 6 weeks, and in 97% within 6 months. Permanent renal impairment never developed after normal urinalysis; it occurred in 1.6% of those with isolated urinary abnormalities, and in 19.5% of those who developed nephritic or nephrotic syndrome.
CONCLUSION: No long term renal impairment occurred after normal urinalysis. Even if urinalysis is normal at presentation, the testing should be continued for six months. There is no need to follow up after the first six months those whose urinalysis remains normal.

PMID 15871983  Arch Dis Child. 2005 Sep;90(9):916-20. doi: 10.1136/adc・・・
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: H Iqbal, A Evans
雑誌名: Arch Dis Child. 2005 Sep;90(9):985-6. doi: 10.1136/adc.2004.061598.
Abstract/Text
PMID 16113142  Arch Dis Child. 2005 Sep;90(9):985-6. doi: 10.1136/adc.・・・
著者: D Pyne, R Mootoo, A Bhanji
雑誌名: Rheumatology (Oxford). 2001 Dec;40(12):1430-1.
Abstract/Text
PMID 11752528  Rheumatology (Oxford). 2001 Dec;40(12):1430-1.
著者: Jaana Ronkainen, Olli Koskimies, Marja Ala-Houhala, Marjatta Antikainen, Jussi Merenmies, Jukka Rajantie, Timo Ormälä, Juha Turtinen, Matti Nuutinen
雑誌名: J Pediatr. 2006 Aug;149(2):241-7. doi: 10.1016/j.jpeds.2006.03.024.
Abstract/Text OBJECTIVE: To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Schönlein purpura (HSP) in a placebo-controlled trial.
STUDY DESIGN: A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis.
RESULTS: Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024).
CONCLUSIONS: The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.

PMID 16887443  J Pediatr. 2006 Aug;149(2):241-7. doi: 10.1016/j.jpeds.・・・
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: 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: Marco Zaffanello, Milena Brugnara, Massimo Franchini
雑誌名: ScientificWorldJournal. 2007 Jan 10;7:20-30. doi: 10.1100/tsw.2007.23. Epub 2007 Jan 10.
Abstract/Text Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis.

PMID 17221139  ScientificWorldJournal. 2007 Jan 10;7:20-30. doi: 10.11・・・
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: J I Shin, J M Park, Y H Shin, J H Kim, J S Lee, H J Jeong
雑誌名: Scand J Rheumatol. 2005 Sep-Oct;34(5):392-5. doi: 10.1080/03009740510026544.
Abstract/Text OBJECTIVE: To clarify the therapeutic role of cyclosporin A (CyA) for patients with Henoch-Schönlein purpura nephritis (HSPN) showing nephrotic-range proteinuria.
METHODS: The clinical and histological findings of eight children (7.7+/-3.8 years), who were treated with CyA and prednisolone, were evaluated retrospectively. All underwent a renal biopsy before therapy, and six of the eight patients received a follow-up biopsy after therapy.
RESULTS: The histological grade of the International Study of Kidney Disease in Children (ISKDC) was improved in all six patients who received a follow-up biopsy (pre-therapy, four grade IIIa and two grade IIIb; post-therapy, one grade I and five grade II) and it was statistically significant (p = 0.031). The activity index was significantly decreased after therapy (8.3+/-1.6 vs. 3.5+/-1.5, p = 0.031), and the chronicity index (0.5+/-0.5 vs. 0.7+/-1.0) and tubulointerstitial (TI) scores (1.5+/-1.3 vs. 0.8+/-1.6) did not change. There was a reduction in proteinuria from 3.2+/-2.3 to 0.1+/-0.1 g/m2/day (p = 0.008) and renal function remained normal in all patients after therapy. However, one patient showed CyA-induced nephrotoxicity at a second biopsy. After an average follow-up period of 3.8 years, six patients showed normal urine and renal function, and two showed minor urinary abnormalities.
CONCLUSION: This study suggests that CyA therapy is effective in reducing proteinuria, which is a known risk factor for the development of renal insufficiency in HSPN and may regress the renal pathology in patients with nephrotic-range proteinuria.

PMID 16234188  Scand J Rheumatol. 2005 Sep-Oct;34(5):392-5. doi: 10.10・・・
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: J T Flynn, W E Smoyer, T E Bunchman, D B Kershaw, A B Sedman
雑誌名: Am J Nephrol. 2001 Mar-Apr;21(2):128-33. doi: 46235.
Abstract/Text BACKGROUND: Henoch-Schönlein Purpura (HSP) is a common childhood vasculitis with manifestations in numerous organ systems, including glomerulonephritis. Patients with more severe HSP-associated glomerulonephritis may develop chronic renal failure. Currently, no widely accepted treatment protocols exist for patients with significant renal involvement.
METHODS: We retrospectively reviewed the clinical courses of 12 children (mean age 9 years) with HSP glomerulonephritis treated with high-dose corticosteroids plus oral cyclophosphamide. All patients had nephrotic-range proteinuria, and all had significant histopathologic changes on biopsy, including crescentic nephritis in 10 patients. Treatment consisted of either intravenous pulse methylprednisolone or oral prednisone followed by oral cyclophosphamide (2 mg/kg/day) for 12 weeks, along with either daily or alternate-day oral prednisone. Prednisone was tapered following completion of cyclophsophamide.
RESULTS: Serum albumin rose significantly after treatment from 2.8 +/- (SD) 0.5 to 3.7 +/- 0.4 g/dl (p < 0.001), and there was a concurrent reduction in proteinuria, as reflected by decreasing serial protein-to-creatinine ratios: from 6.3 +/- 4.4 to 0.8 +/- 0.8 (p = 0.002). Renal function remained normal in all patients. Hypertension developed during treatment in 10 patients, all but 1 of whom were normotensive at last follow-up, 35 +/- 17 months following biopsy.
CONCLUSIONS: We conclude that treatment of children with HSP nephritis with high-dose corticosteroids plus oral cyclophosphamide is safe and, as in nephrotic syndrome, appears to significantly reduce proteinuria which is a known risk factor for the development of renal insufficiency in HSP. Further studies with larger numbers of patients should be conducted to confirm this finding.

Copyright 2001 S. Karger AG, Basel
PMID 11359020  Am J Nephrol. 2001 Mar-Apr;21(2):128-33. doi: 46235.
著者: Hiroshi Tanaka, Koichi Suzuki, Tohru Nakahata, Etsuro Ito, Shinobu Waga
雑誌名: Pediatr Nephrol. 2003 Apr;18(4):347-50. doi: 10.1007/s00467-003-1094-4. Epub 2003 Mar 28.
Abstract/Text Nine Japanese children with severe proteinuric Henoch-Schönlein purpura nephritis (HSPN) received prompt initiation of oral prednisolone (1.5 mg/kg/day) combined with an 8-week course of cyclophosphamide (2 mg/kg/day) therapy. All underwent renal biopsy before and after treatment. At presentation, urine protein excretion and histologic indices of the mean activity index, the mean chronicity index and the tubulointerstitial (TI) scores in the patients were 5.0+/-1.4, 4.7+/-1.0, 3.9+/-1.6 and 3.7+/-0.5 g/day, respectively. Urine protein excretion, the activity index and the TI scores decreased significantly at the second renal biopsies obtained at a mean interval of 23 months after the first [0.3+/-0.3, 2.4+/-0.5 and 1.4+/-0.7 g/day ( P<0.01), respectively], while the chronicity index did not change. At the latest observation (mean interval 78 months), all except two showed negative proteinuria while no patient showed renal impairment. Although this case series is without controls, our experience suggests that early treatment with oral prednisolone and cyclophosphamide may be beneficial to a proportion of patients with severe proteinuric HSPN.

PMID 12700960  Pediatr Nephrol. 2003 Apr;18(4):347-50. doi: 10.1007/s0・・・
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: J Bergstein, J Leiser, S P Andreoli
雑誌名: Clin Nephrol. 1998 Jan;49(1):9-14.
Abstract/Text The benefits of treating severe Henoch-Schoenlein Purpura (HSP) glomerulonephritis have not been established. In this study, we evaluate the outcome of 21 children with severe HSP nephritis treated with corticosteroids and azathioprine. Between 1977 and 1995, 78 children (age range 1 to 16 years) were seen for evaluation of HSP. Thirty-one underwent kidney biopsy; indications included nephritic and/or nephrotic onset (15 patients), persistently decreased creatinine clearance (5 patients), or proteinuria > 4 g/24 h (11 patients). Twenty treated patients had diffuse mesangial proliferation with crescents in 6-100% (mean 40%) of glomeruli. One treated patient, not biopsied due to extreme obesity, had a creatinine clearance of 49 ml/min/1.73 m2 and proteinuria of 21.3 g/24 h. These 21 patients were initially treated with azathioprine and daily oral prednisone (13 patients) or i.v. methyl-prednisolone (8 patients), followed by azathioprine and alternate-day prednisone for 9-24 (mean 15) months. The average follow-up was 32 months. Over the course of follow-up, 19 treated patients showed a decline in hematuria (> 5 red blood cells/high power field) from 100% to 16% (p < 0.01), a fall in the serum creatinine from 1.71 +/- 2.20 to 0.78 +/- 0.25 mg/dl (p < 0.01), an increase in creatinine clearance from 76 +/- 43 to 122 +/- 26 ml/min/1.73 m2 (p < 0.01), and a reduction in proteinuria from 8.8 +/- 7.5 to 0.47 +/- 0.39 g/24 h (p < 0.01). Two treated patients progressed to end-stage renal failure. There was no difference in outcome comparing patients initially treated with prednisone versus methyl-prednisolone. These observations suggest that corticosteroid and azathioprine therapy is effective in crescentic HSP nephritis.

PMID 9491279  Clin Nephrol. 1998 Jan;49(1):9-14.
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: H Fukui, H Kamitsuji, T Nagao, K Yamada, J Akatsuka, M Inagaki, S Shike, Y Kobayashi, K Yoshioka, S Maki
雑誌名: Thromb Res. 1989 Dec 15;56(6):667-75.
Abstract/Text Arthral, abdominal and renal symptoms in Henoch-Schönlein purpura (HSP) were scored. Coagulation factor XIII (F XIII) activity was determined in fifty-six children with HSP and the correlation with the severity score of the clinical symptoms was investigated. As a result, it was found that the decrease in F XIII level was correlated with the severity score of clinical symptoms, particularly abdominal symptoms. Based on the results, a controlled study was performed in 24 cases with moderate symptoms divided into a group treated with F XIII concentrate and a non-treated group to investigate clear-cut efficacy as a next study. In three days after the administration the symptoms were improved remarkably in accordance with the increase of F XIII level compared with non-treated group and scoring of clinical symptoms was confirmed to be useful for assessing the application of the F XIII concentrate to HSP.

PMID 2699100  Thromb Res. 1989 Dec 15;56(6):667-75.

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