CREST 증후군

CREST 증후군
다른 이름Calcinosis-Raynaud phenomenon-esophageal involvement-sclerodactyly-telangiectasia syndrome[1]
CREST 증후군 (석회증과 손발가락경화증)
진료과류마티스내과

제한형 전신경화증(limited cutaneous form of systemic sclerosis, IcSSc)으로도 불리는 CREST 증후군은 신체의 여러 계통을 침범하는 결합조직질환의 일종이다. 'CREST'라는 이름은 질환에서 나타나는 5개의 특징, 즉 석회증(calcinosis), 레이노 현상(Raynaud's phenomenon), 식도운동장애(sophageal motility disorder), 손발가락경화증(sclerodactyly), 모세혈관확장증(telangiectasia)을 줄인 것이다.[2]

CREST 증후군은 동원체에 대한 자가항체와 연관되어 있으며, 전신피부경화증에서 자주 침범하는 콩팥은 보통 침범하지 않는다. 폐를 침범하는 경우 폐동맥고혈압의 일종으로 나타난다.

같이 보기

각주

  1. ↑ RESERVED, INSERM US14-- ALL RIGHTS. “Orphanet: CREST syndrome”. 《www.orpha.net》 (영어). 2019년 8월 3일에 원본 문서에서 보존된 문서. 2019년 4월 27일에 확인함. 
  2. ↑ Winterbauer RH (1964). “Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia”. 《Bulletin of the Johns Hopkins Hospital》 114: 361–83. PMID 14171636. 

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