Pathology and Pathobiology of Pulmonary Hypertension

被引：95

作者：

Guignabert, Christophe ^{[1
,4
]}

Dorfmuller, Peter ^{[1
,2
,3
,4
]}

机构：

[1] INSERM, Dept Cardiovasc Res, LabEx LERMIT, UMR S 999, Le Plessis Robinson, France

[2] Ctr Chirurg Marie Lannelongue, Dept Pathol, Le Plessis Robinson, France

[3] Ctr Chirurg Marie Lannelongue, Res Dept, Le Plessis Robinson, France

[4] Univ Paris Sud, DHU Thorax Innovat TORINO, Sch Med, F-92350 Le Plessis Robinson, France

来源：

SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE | 2013年 / 34卷 / 05期

关键词：

pulmonary arterial hypertension; pulmonary vascular remodeling; endothelial dysfunction; cellular cross talk; inflammation; BMPR2; ANTIENDOTHELIAL CELL ANTIBODIES; RESISTANT ENDOTHELIAL-CELLS; ARTERIAL-HYPERTENSION; IMMUNOSUPPRESSIVE THERAPY; SIGNALING CONTRIBUTES; LYMPHOID NEOGENESIS; PLEXIFORM LESIONS; VASCULAR DISEASE; PATHOGENESIS; PROLIFERATION;

D O I：

10.1055/s-0033-1356496

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

Pulmonary hypertension is a devastating, life-threatening disorder with no curative options, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Although the etiologies of pulmonary arterial hypertension are multiple and its pathogenesis is complex, there is growing evidence that inflammation, endothelial dysfunction, aberrant vascular wall cell proliferation, as well as mutations in the bone morphogenetic protein receptor type 2 gene play a crucial role in triggering pathological vascular remodeling. The present article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

引用

页码：551 / 559

页数：9

共 79 条

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