현명내과

의학적 검토: 정지인 내과 전문의 / 류마티스내과 분과전임의 (서울대학교병원 전임의 과정 수료 (2021-2024))

영남대학교 의과대학 내과학 석박사 수료

최종 업데이트: 2026-04-22

개요

전신경화증(SSc, ICD-10: M34.9)은 피부와 내부 장기의 섬유화, 혈관병증, 면역 이상을 특징으로 하는 자가면역 결합조직 질환입니다. 제한형(lcSSc, 항centromere)과 미만형(dcSSc, 항SCL-70)으로 분류되며, 폐섬유화와 폐동맥고혈압이 주요 사망원인입니다.

원인과 위험인자

분류세부 인자기전/특징
자가면역항SCL-70, 항centromere, 항RNA polymerase III하위 유형 결정
혈관병증내피세포 손상 → 혈관 리모델링레이노 현상, 디지털 궤양
섬유화TGF-β, CTGF 과다 → 콜라겐 축적피부 경화, 장기 섬유화
유전+환경HLA 연관, 실리카/용매 노출유전-환경 상호작용

증상

진단

ACR/EULAR 2013 분류기준

피부 경화(MCP 근위부), 손가락 부종/경화, 손가락 끝 궤양/반흔, 모세혈관확장, 레이노, SSc 관련 자가항체, 폐동맥고혈압, ILD 등 가중치 합산.

자가항체

항centromere(제한형 → PAH 위험), 항SCL-70/topoisomerase I(미만형 → ILD 위험), 항RNA pol III(신장위기 위험).

치료

장기별 치료

ILD: mycophenolate(SLS-II trial, 1차), nintedanib(SENSCIS trial), cyclophosphamide. PAH: ERA+PDE5i 병합 ± prostanoid(AMBITION trial). 신장위기: ACEi 즉시 투여(ARB 아님). GERD: PPI. 레이노/궤양: CCB, iloprost.

피부 경화

미만형 초기: MTX, mycophenolate. 중증: 자가조혈모세포이식(ASTIS, SCOT trial).

PubMed 근거 요약

ACR/EULAR 2013 SSc 분류기준이 진단의 국제 표준입니다. SLS-II(Tashkin et al., 2016, Lancet Respir Med)에서 mycophenolate가 cyclophosphamide와 동등한 ILD 효과를 보이면서 부작용이 적었습니다. SENSCIS(Distler et al., 2019, NEJM)에서 nintedanib이 SSc-ILD의 FVC 감소를 유의하게 억제했습니다.

현명신경외과 류마티스내과의 접근

현명신경외과 류마티스내과에서는 전신경화증의 포괄적 진단(피부, 폐, 심장, 신장, 위장관), 자가항체 기반 예후 평가, 장기별 맞춤 치료를 전문적으로 제공합니다.

자주 묻는 질문

Q: 전신경화증은 피부만 딱딱해지는 병인가요?

A: 피부 경화가 대표 증상이지만, 폐(간질성 폐질환, 폐동맥고혈압), 소화기(역류, 연하곤란), 신장(경피증 신위기) 등 내장 장기를 침범하는 전신 질환입니다.

Q: 전신경화증은 치료가 가능한가요?

A: 완치는 어렵지만, 장기별 합병증을 적극 관리하여 진행을 늦출 수 있습니다. 폐섬유화에는 닌테다닙·미코페놀레이트, 폐동맥고혈압에는 혈관확장제를 사용합니다.

Q: 전신경화증(경피증)의 초기 증상은 무엇인가요?

A: 레이노 현상(손가락 3색 변화)이 약 90%에서 가장 먼저 나타납니다. 이후 손가락 부종, 피부 경화, 삼킴 곤란이 진행됩니다. 항Scl-70, 항세포질항체 등으로 분류합니다.

Q: 전신경화증의 폐 합병증은 왜 중요한가요?

A: 간질성 폐질환(ILD)과 폐동맥 고혈압(PAH)이 주요 사망 원인입니다. HRCT와 폐기능검사로 조기 발견하며, 닌테다닙, 미코페놀레이트가 ILD 진행 억제에 효과적입니다.

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함께 읽어보기

본 콘텐츠는 의학 논문과 임상 가이드라인을 기반으로 작성되었으며, 개인의 의료 상황에 따라 다를 수 있습니다. 정확한 진단과 치료는 전문의 상담을 통해 결정하시기 바랍니다.