ヘノッホ・シェーンライン紫斑病。リウマチ性紫斑病
WordNet
- a person suffering with rheumatism
- any of several blood diseases causing subcutaneous bleeding (同)peliosis
PrepTutorEJDIC
- リューマチの / リューマチにかがった / リューマチ患者
UpToDate Contents
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English Journal
- A Case of Acute Rheumatic Fever With Henoch Schonlein Purpura.
- Bostan Gayret O1, Erol M1, Yigit O1, Kutanis E1, Yavuz S1, Muhammedoglu A2, Kasapcopur O3.
- Iranian journal of pediatrics.Iran J Pediatr.2015 Dec;25(6):e1092. Epub 2015 Dec 23.
- PMID 26635933
- Autoimmune diseases in Adult Life after Childhood Cancer in Scandinavia (ALiCCS).
- Holmqvist AS1, Olsen JH2, Mellemkjaer L2, Garwicz S1, Hjorth L1, Moëll C1, Månsson B3, Tryggvadottir L4, Hasle H5, Winther JF2; ALiCCS study group.
- Annals of the rheumatic diseases.Ann Rheum Dis.2015 Nov 10. pii: annrheumdis-2015-207659. doi: 10.1136/annrheumdis-2015-207659. [Epub ahead of print]
- OBJECTIVES: The pattern of autoimmune diseases in childhood cancer survivors has not been investigated previously. We estimated the risk for an autoimmune disease after childhood cancer in a large, population-based setting with outcome measures from comprehensive, nationwide health registries.METHOD
- PMID 26555403
- Final diagnoses of children and adolescents with musculoskeletal complaints.
- Cavkaytar O1, Düzova A, Tekşam O, Karabulut E, Derman O, Kale G, Ozen S.
- Minerva pediatrica.Minerva Pediatr.2015 Sep 10. [Epub ahead of print]
- AIM: Musculoskeletal complaints (MSCs) are one of the leading causes of outpatient admissions; however analytical and epidemiological data are limited. The aim of this study is to identify the etiology of MSCs (excluding acute traumatic conditions) in children and adolescents, and to identify clues
- PMID 26365744
Japanese Journal
- Cryofibrinogenemia Associated with Sjoegren's Syndrome: A case of Successful Treatment with High-dose Corticosteroid
- Yoshida Ken,Yokoyama Toru,Toyokawa Yasuhiko,Yasuda Jun,Kingetsu Isamu,Kurosaka Daitaro,Yamada Akio
- Internal Medicine 46(13), 1039-1042, 2007
- … Cryofibrinogenemia (CF) has not been often reported as a complication of various rheumatic diseases. … We describe a 44-year-old woman with CF associated with Sjögren's syndrome (SS), who developed digital necrotic ulcerations and purpura of the lower legs. …
- NAID 130000076164
- A Patient with Wegener's Granulomatosis with Initial Clinical Presentations of Henoch-Schonlein Purpura
- MIYATA Masayuki,KANNO Kaori,NISHIMAKI Tomoe,SAKUMA Fumitaka,IWATSUKI Keiji,KASUKAWA Reiji
- Internal medicine 40(10), 1050-1054, 2001-10
- … The initial presentation of a patient with Wegener's granulomatosis was indistinguishable from that of Henoch-Schönlein purpura. … The patient presented with skin purpura and pulmonary hemorrhage followed by purpura in the colon. … The diagnosis of this patient at that time was Henoch-Schönlein purpura. …
- NAID 10007002711
- A Patient with Graves' Disease who Developed Hypothyroidism Associated with Thyroid Stimulation Blocking Immunoglobulin during Anti-thyroid Drug Therapy.
- INOMATA HIROAKI,TSUYUSAKI TOSHIYUKI,SASAKI NOZOMU,TAMARU KIYOE,NIIMI HIROO,NAKAJIMA HIRONORI
- Endocrinologia Japonica 35(3), 379-384, 1988
- … A girl, 12 years of age, developed Graves' disease compounded with rheumatic fever and idiopathic thrombocytopenic purpura. … Thrombocytopenia improved under short-term treatment with steroids and her mitral valvular insufficiency, due to the rheumatic fever, disappeared 4 years later. …
- NAID 130000952925
Related Links
- Rheumatic Purpura and Peliosis Rheumatica. James Metcalfe. Full text. Full text is available as a scanned copy of the original print version. Get a printable copy ( PDF file) of the complete article (297K), or click on a page image below to ...
- OBJECTIVE: To define the clinical manifestations, autoantibody associations, optimal treatment, and prognosis of hypergammaglobulinemic purpura associated with systemic autoimmune rheumatic diseases. METHODS: Of 303 consecutive ...
★リンクテーブル★
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- 英
- IgA vasculitis IgAV
- 同
- アナフィラクトイド紫斑病、ヘノッホ・シェーンライン紫斑病 Henoch-Schönlein purpura, Henoch-Schonlein purpura, HSP, Schonlein-Henoch purpura, Schonlein-Henoch purpura
- ヘノッホ・シェンライン紫斑病、Henoch-Schonlein紫斑病、Henoch-Schonlein紫斑、シェンライン・ヘノッホ紫斑病、シェーンライン・ヘノッホ紫斑病、Schonlein-Henoch紫斑病
- アナフィラキシー様紫斑病 アナフィラクトイド紫斑病 アナフィラクトイド紫斑、anaphylactoid purpura、アレルギー性紫斑病 アレルギー性紫斑 allergic purpura purpura allergica、アレルギー性血管炎 allergic vasculitis、リウマチ性紫斑病 rheumatic purpura purpura rheumatica、ヘノッホ紫斑病
- 関
- IgA腎症。血管性紫斑病、皮膚小血管性血管炎、血管炎、劇症紫斑病、血小板非減少性紫斑病
まとめ
- 先行する上気道感染後に出現する紫斑が特徴的な血管炎である。IgAによる免疫複合体の血管壁への沈着、およびこれに次いで起こるIII型アレルギーが本態と考えられている。症状はG2AP、すなわち消化管症状、糸球体腎炎、関節痛、紫斑である。血液検査では軽度の好中球増多・好酸球増多がみられ、血液中のIgAも上昇するが、血小板、補体、IgG,IgEは変化しない。
定義
- CHCC2012
- IgA1優位の免疫沈着を伴い、小血管(主に毛細血管、細静脈または細動脈)を障害する血管炎。しばしば、皮膚、消化管、そして高頻度に関節炎を来す。IgA腎症と区別できない糸球体腎炎を生ずる。
概念
- 全身性疾患
- 血管炎(血管性紫斑病の一つである) ← 皮膚小血管性血管炎の特殊型
- 二次的にIgAによる免疫複合体がメサンギウムに沈着し糸球体腎炎を引き起こす
病因
- IgA免疫複合体が血管壁に沈着。III型アレルギーの機序により、補体や好中球による組織障害
- 1. 溶連菌感染
- 2. 薬剤(ペニシリン、アスピリン)、食物(牛乳、卵)
疫学
病理
症状 (PED.805, NDE.137)
- 皮膚(purpuric rash)、消化管(abdominal pain)、関節(arthritis)、腎臓(glomerulonephritis)
- HSPの症状は「GA2P」と覚えてみる?
- ほぼ全例
- 両側の下腿や足背を中心に、時には大腿~上肢~腹部にまで、直径数~10mm以内の浸潤をふれる点状出血・紫斑が播種状に生じる。ときに圧痛が見られる。(NDE.138)
- 丘疹状で触知可能(palpable purpura)
[show details]
- http://graphics8.nytimes.com/images/2007/08/01/health/adam/19832.jpg
- http://www.umm.edu/graphics/images/en/19831.jpg
- http://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura
- 1/3の症例
- 一過性の限局性浮腫。東部、顔面、手足、陰嚢
消化管
- 約半数~80%
- 腹部の疝痛様疼痛、悪心や嘔吐、吐血、下血
関節
- 約2/3の症例
- 足、膝、手、肘などの関節炎症状。関節の腫脹、疼痛
腎臓
- 発症から一ヶ月以内人タンパク尿、血尿で始まる腎炎を合併。多くは顕微鏡的血尿。
- 急性腎炎~ネフローゼ
- 組織的にはメサンギウム増殖性腎炎を呈する。
診断
検査
- PED.805
- 血小板:正常~やや増加 ← 血管炎が紫斑の原因であって、血小板減少が原因ではない。
- PED.805
治療
国試
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- 関
- rheumatica、rheumatism、rheumatoid
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