WordNet
- (physical chemistry) a sample of matter in which substances in different phases are in equilibrium; "in a static system oil cannot be replaced by water on a surface"; "a system generating hydrogen peroxide"
- instrumentality that combines interrelated interacting artifacts designed to work as a coherent entity; "he bought a new stereo system"; "the system consists of a motor and a small computer"
- a group of independent but interrelated elements comprising a unified whole; "a vast system of production and distribution and consumption keep the country going" (同)scheme
- a complex of methods or rules governing behavior; "they have to operate under a system they oppose"; "that language has a complex system for indicating gender" (同)system of rules
- a group of physiologically or anatomically related organs or parts; "the body has a system of organs for digestion"
- a procedure or process for obtaining an objective; "they had to devise a system that did not depend on cooperation"
- the living body considered as made up of interdependent components forming a unified whole; "exercise helped him get the alcohol out of his system"
- gradually advancing in extent
- a tense of verbs used in describing action that is on-going (同)progressive tense, imperfect, imperfect tense, continuous tense
- favoring or promoting reform (often by government action) (同)reformist, reform-minded
- (of a card game or a dance) involving a series of sections for which the participants successively change place or relative position; "progressive euchre"; "progressive tournaments"
- (of taxes) adjusted so that the rate increases as the amount of income increases
- advancing in severity; "progressive paralysis"
- favoring or promoting progress; "progressive schools"
- affecting an entire system; "a systemic poison"
- any pathological hardening or thickening of tissue (同)induration
PrepTutorEJDIC
- 〈C〉(関連した部分から成る)『体系』,系統,組織[網],装置 / 〈C〉(教育・政治などの)『制度』,機構;《the~》体制 / 〈C〉(思想・学問などの)『体系』,学説 / 〈C〉(…の)『方法』,方式,やり方《+of doing》 / 〈U〉正しい方針(筋道,順序) / 〈U〉《the~》(身体の)組織,系統 / 〈U〉《the~,one's~》身体,全身
- (行列などが)『前進する』,進んで行く / (事態が)進展する,進行する / 『進歩的な』,革新的な / (病気・暴力などが)次第に悪くなる(広がる) / (課税が)累進的な / (文法で)進行[形]の / 進歩的な人,革新主義者
- (身体・言語のように)体系の,組織の / 全身の,全身的
- (組織・血管などの)硬化,硬化症
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/08/18 18:30:23」(JST)
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- 1. 成人における全身性硬化症（強皮症）の臨床症状の概要 overview of the clinical manifestations of systemic sclerosis scleroderma in adults
- 2. 強皮症の概要および分類 overview and classification of scleroderma disorders
- 3. Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults
- 4. 成人における全身性硬化症（強皮症）の診断および鑑別診断 diagnosis and differential diagnosis of systemic sclerosis scleroderma in adults
- 5. 全身性硬化症（強皮症）における肺合併症の概要 overview of pulmonary complications of systemic sclerosis scleroderma
English Journal
- Systemic sclerosis with multiple nodules: characterization of the extracellular matrix.
- Moinzadeh P, Agarwal P, Bloch W, Orteu C, Hunzelmann N, Eckes B, Krieg T.SourceDepartment of Dermatology, University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany, pia.moinzadeh@uk-koeln.de.
- Archives of dermatological research.Arch Dermatol Res.2013 Sep;305(7):645-52. doi: 10.1007/s00403-013-1383-0. Epub 2013 Jul 9.
- Systemic sclerosis (SSc) is still an enigmatic disease of unknown etiology, although the pathophysiology is thought to be based on vascular alterations as well as immunological and fibrotic processes. Here we present the case of a female patient with diffuse SSc (dSSc), who developed multiple subcut
- PMID 23836353
- Systemic sclerosis and cryoglobulinemia: Our experience with overlapping syndrome of scleroderma and severe cryoglobulinemic vasculitis and review of the literature.
- Giuggioli D, Manfredi A, Colaci M, Manzini CU, Antonelli A, Ferri C.SourceChair and Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy. Electronic address: giuggioli.dilia@policlinico.mo.it.
- Autoimmunity reviews.Autoimmun Rev.2013 Sep;12(11):1058-63. doi: 10.1016/j.autrev.2013.06.013. Epub 2013 Jun 24.
- OBJECTIVE: Systemic sclerosis (SSc) is an immune-mediated disorder characterized by multiple organ fibrotic alterations and diffuse microangiopathy. The SSc can be associated with other connective tissue diseases and less frequently with systemic vasculitides, including cryoglobulinemic vasculitis (
- PMID 23806565
- Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face.
- Careta MF, Leite Cda C, Cresta F, Albino J, Tsunami M, Romiti R.SourceDepartment of Dermatology, University of Sao Paulo Medical School, Brazil. Electronic address: mfcareta@yahoo.com.br.
- Autoimmunity reviews.Autoimmun Rev.2013 Sep;12(11):1064-9. doi: 10.1016/j.autrev.2013.05.005. Epub 2013 Jun 19.
- INTRODUCTION: Scleroderma featuring rare connective tissue disease that manifests as skin sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement and localized scleroderma or morphea which
- PMID 23791631
Japanese Journal
- 急速に進行した強皮症合併肺動脈性肺高血圧症の一例
- 石田 素子,宮村 知也,海江田 智絵,木村 大作,中村 彰宏,高濱 宗一郎,喜安 純一,南 留美,山本 政弘,末松 栄一
- 日本臨床免疫学会会誌 36(3), 170-174, 2013
- … 症例は68歳女性．平成10年頃からRaynaud症状，平成20年頃より指尖部潰瘍が出現し，増悪消退を繰り返していた．平成24年1月当院初診し，強指症状，両肘を超えない皮膚硬化，短指症，抗centromere抗体陽性で強皮症（limited cutaneous systemic sclerosis ; …
- NAID 130003364175
- A case of slowly progressive scleroderma kidney
- OKABE Masahiro,TSUBOI Nobuo,SUZUKI Takahide,YOKOO Takashi,MIYAZAKI Yoichi,UTSUNOMIYA Yasunori,OHNO Iwao,HOSOYA Tatsuo
- Clinical and experimental nephrology 15(3), 430-433, 2011-06-01
- NAID 10029005972
- A case of progressive digital ischemia after early withdrawal of gemcitabine and S-1 in a patient with systemic sclerosis.
- Zaima C,Kanai M,Ishikawa S,Kawaguchi Y,Masui T,Mori Y,Nishimura T,Matsumoto S,Yanagihara K,Chiba T,Mimori T
- Japanese journal of clinical oncology 41(6), 803-806, 2011-06-00
- … The safety of chemotherapy for patients with systemic sclerosis is unclear, and there are few published reports documenting the side effects of chemotherapy in patients with this condition. … Here, we report the case of a patient with systemic sclerosis who developed severe digital ischemia during combination gemcitabine/S-1 chemotherapy for pancreatic cancer. …
- NAID 120004225524
Related Links
- Progressive systemic sclerosis is a generalized disorder of connective tissue in which there is thickening of dermal collagen bundles, and fibrosis and vascular abnormalities in internal organs.
- sclerosis /scle·ro·sis/ (-ro´sis) an induration or hardening, especially from inflammation and in diseases of the interstitial substance; applied chiefly to such hardening of the nervous system or to hardening of the blood vessels.
Related Pictures
★リンクテーブル★
| リンク元 | 「全身性強皮症」「PSS」「進行性全身性強皮症」 |
| 関連記事 | 「systemic」「system」「progressive」 |
「全身性強皮症」
- 英
- systemic sclerosis, systemic scleroderma, SSc
- 同
- 汎発性強皮症 diffuse sclerosis、進行性全身性硬化症 progressive systemic sclerosis PSS、全身性硬化症
- 関
- 強皮症、膠原病
- 難病
概念
- 皮膚硬化を特徴とする強皮症のうち、皮膚のみでなく全身の諸臓器(肺、消化管、心、腎、関節など)に病変がみられるもの。
病因
- 遺伝
- 環境因子
- 珪肺症患者：リスク110倍
- 美容などの豊胸手術後に発症：シリコンがアジュバンドとして作用
- 塩化ビニル工場従事者に多い
- 薬剤(ブレオマイシン、トリプトファン)
- マイクロキメリズム(胎児由来血液幹細胞が母胎に移行)
疫学
- 30-50歳に好発。男女比は1：3-4。(NDE.171)
病型
- limited cuteneous SSc, diffuse cuteneous SSc
limited cuteneous SSc
- 皮膚硬化は肘から末梢に限局
- 内臓病変：軽度
- 予後：良好
- 自己抗体：抗セントロメア抗体
CREST症候群
- 全身性強皮症の一亜型
- C:carcinosis:石灰沈着
- R:Raynaud's phenomenon:レイノー現象
- E:esophageal dysfunction:食道機能不全
- S:sclerodacrylia:強指症
- T:teleangiectasia:毛細血管拡張
diffuse cuteneous SSc
- 皮膚硬化は肘から近位
- 内臓病変：急速に進行
- 予後：不良
- 自己抗体：抗Scl-70抗体
症状
- 初発症状：レイノー現象、指、手の硬化
- 関節炎、食道蠕動運動低下、下部食道の拡張、肺線維症(55%)、肺高血圧(5%)、心症状(不整脈、伝導障害)(10-20%)、心膜炎(3%)、吸収不良症候群、強皮腎(悪性高血圧症。5%)、橋本甲状腺炎
- 強皮腎：血管内皮細胞の障害→血管内膜の肥厚、内腔の狭窄→血管の攣縮・虚血→輸入動脈、糸球体係蹄の壊死→レニン産生の亢進→悪性高血圧→急性腎不全
症状の出現頻度
- 100%：皮膚硬化、レイノー現象
- 60%：食道機能障害、肺線維症
- 20%：小腸、大腸、ミオパチー
- 10%：心肥大
- 5%：肺高血圧、強皮腎
| 病態 | レイノー現象 | 抗核抗体 | リウマトイド因子 | 抗好中球細胞質抗体 | 皮疹 | 皮下結節 | 関節炎 | 筋炎 | 漿膜炎 | 自己抗体 | |
| Scl-70 | |||||||||||
| 病理 | 壊死性血管炎 | 糸球体腎炎 | 間質性肺炎 | 心炎 | 唾液腺炎 | オニオンスキン病変 | ワイヤーループ病変 | ヘマトキシリン体 | LE細胞 | ||
検査
診断
(1) 大基準 手指あるいは足趾を越える皮膚硬化※１ (2) 小基準 ① 手指あるいは足趾に限局する皮膚硬化 ② 手指尖端の陥凹性瘢痕，あるいは指腹の萎縮※２ ③ 両側性肺基底部の線維症 ④ 抗トポイソメラーゼⅠ（Scl-70）抗体または抗セントロメア抗体陽性 (3) 除外基準 ① ※1 限局性強皮症（いわゆるモルフィア）を除外する ② ※2 手指の循環障害によるもので，外傷などによるものを除く (4) 診断の判定 大基準を満たすものを強皮症と診断する。 大基準を満たさない場合は，小基準の①かつ②～④のうち１項目以上を満たすものを強皮症と判断する
治療
- ステロイド → 皮膚の初期病変に対しては浮腫を除いて無効
- 皮膚硬化：D-ペニシラミン
- 肺線維症：ステロイド、IFN-γ、シクロホスファミド、シクロスポリン
- 消化管病変：PPI、(逆流性食道炎)H2ブロッカー、(吸収不良症候群)カナマイシン
- 関節炎：NSAID、ステロイド
- レイノー現象：血管拡張薬(カルシウム拮抗薬、PGI2製剤、PGE1製剤)
- 肺高血圧：血管拡張薬(カルシウム拮抗薬、PGI2製剤、PGE1製剤)
- 強皮腎：ACE阻害薬
- 進行性腎不全に陥り予後は重大であるが、早期のACE阻害薬による治療が予後を改善させた(REU.195)
予後
- 5年生存率 ：93.7%
- 10年生存率 :76.6%
予後因子
- 全身の皮膚硬化
- 腎病変
- 心、血管病変
- 抗Scl-70抗体 ←予後不良
- 抗セントロメア抗体 ←予後良好
「PSS」
「進行性全身性強皮症」
「systemic」
- adj.
- 全身性の、全身的な、全身の、(植物)浸透移行性の
「system」
- n.
「progressive」
- adj.
- 進行性の