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폐동맥고혈압의 병인 및 병태생리

Pathogenesis and pathophysiology of pulmonary arterial hypertension

장성아
대한내과학회 (The Korean Association Of Internal Medicine)|2010.01|대한내과학회지 (The Korean Journal of Medicine)|제78권 제1호|pp. 14-19

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키워드

폐동맥고혈압; 병인; 병태생리Pulmonary arterial hypertension; Pathogenesis; Pathophysiology
주제분류내과학

초록

폐동맥고혈압은 폐동맥 자체의 병적인 변화로 인하여 폐동맥압력과 이에 따른 우심실 부전을 가져오는 질환이다. 폐동맥고혈압의 발생기전은 명확하지 않으나 환자가 가지고있는 유전적 소인, 기저 질환에 더하여 환경적 자극에 의한세포내외의 신호전달 체계의 변화가 복합적으로 영향을 미치는 것으로 보인다. 폐동맥고혈압의 병인을 이해하는 것은기존 폐동맥고혈압 특이 약제(pulmonary arterial hypertension specific drug)를 이해하고, 새로운 약제의 개발하는데 이론적바탕을 제공한다. 폐동맥고혈압에 대한 이해가 높아질수록폐동맥고혈압이 불치의 병에서 치료할 수 있는 만성질환으로 변모하기를 기대해 본다.

Abstract

Pulmonary arterial hypertension (PAH) is a rare disease with progressive pathology in pulmonary arteries. Although we lack full understanding of PAH pathophysiology, a variety of vascular components are known to be involved in the pathogenesis of PAH. Endothelial dysfunction is one of the most important components, resulting in vasoconstriction and thrombus formation in pulmonary arteries. Muscularization of pulmonary artery with neointima formation contributes to the occlusion of pulmonary arteries. Hyperplasia of smooth muscle cells as well as vasoconstriction are associated with abnormal Ca2+ and K+ transport within smooth muscle cells. Plasma level of 5-HT (serotonin) and expression of its transporters are elevated in PAH, which also mediates vasoconstriction and hyperplasia of smooth muscle cells. On top of the changes found in vessel itself, alterations in extracellular matrix and a cell-to-cell interaction also play an important role in PAH pathogenesis. Inflammatory process with associated cytokines is believed to generate vascular remodeling and as such inhibitor of these processes can be a potential treatment target for PAH. Thanks to all of this pathological interplay briefly described above, plexiform lesion develops at the late stage of PAH and clinically a high pulmonary vascular resistance ensues, consequently resulting in right ventricular failure. Given prognostic relevance of pulmonary vascular resistance, the understanding of pathogenesis and pathophysiology of PAH is essential in guiding a window to developing novel treatment targets and finally improving prognosis as well as increasing a quality of life in PAH patients.

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관련 논문AI추천

새로 개정된 폐고혈압의 임상적 분류정해억대한내과학회 · 대한내과학회지2010 · 제78권 제1호폐동맥고혈압의 진단과 평가서혜선, 이내희대한내과학회 · 대한내과학회지2010 · 제78권 제1호유전성 폐동맥고혈압과 관련된 유전자 연구이영재대한내과학회 · 대한내과학회지2010 · 제78권 제1호Current status of pulmonary arterial hypertension in Korea장영우, 정욱진대한내과학회 · The Korean Journal of Internal Medicine2019 · 제34권 제4호중증 폐동맥고혈압이 동반된 만성폐쇄성폐질환 1예김석민, 박찬서, 손창우, 유성근, 이관호, 정진홍, 진현정영남대학교 의과대학 · Journal of Yeungnam Medical Science2008 · 제25권 제1호15 years journey of idiopathic pulmonary arterial hypertension with BMPR2 mutation박수정, 안경진, 장영우, 정욱진대한고혈압학회 · Clinical Hypertension2019 · 제25권 제4호

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