Giordano Nicola, Montella Antonio, Corallo Claudio, Ruocco Gaetano, Chirico Chiara, Palazzuoli Alberto, Nuti Ranuccio, Pecetti Gianluca
Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy.
Medical and Scientific Direction, Actelion Pharmaceuticals, Imola, Italy.
Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S182-9. Epub 2015 Sep 1.
Systemic sclerosis (SSc) is a heterogeneous disorder characterised by dysfunction of the endothelium and dysregulation of fibroblasts, resulting in excessive production of collagen, and abnormalities of the immune system. Progressive fibrosis of the skin and internal organs is a pathologic hallmark of the disease, resulting in major organ damage and failure. Pulmonary hypertension (PH) is frequent in patients with SSc and, pulmonary arterial hypertension (PAH) represents one of the main causes of death. PH is not a specific disease, but a haemodynamic condition characterized by a mean pulmonary pressure ≥25mmHg. In SSc, because of the great variability in clinical manifestation, it is possible to identify pulmonary hypertension due to left heart disease, PH due to respiratory disease or pulmonary arterial hypertension. The knowledge of PH and the right diagnosis are crucial to assess the most appropriate therapeutic strategy. In this article, the new classification criteria of PH have been examined taking into account the SSc clinical evolution and focusing on the different underlying pathogenetic mechanisms.
系统性硬化症（SSc）是一种异质性疾病，其特征为内皮功能障碍和成纤维细胞失调，导致胶原蛋白过度产生以及免疫系统异常。皮肤和内脏器官的进行性纤维化是该疾病的病理标志，可导致主要器官损害和功能衰竭。肺动脉高压（PH）在系统性硬化症患者中很常见，而肺动脉高压（PAH）是主要死因之一。PH并非一种特定疾病，而是一种以平均肺动脉压≥25mmHg为特征的血流动力学状态。在系统性硬化症中，由于临床表现差异很大，有可能识别出由左心疾病引起的肺动脉高压、由呼吸系统疾病引起的PH或肺动脉高压。了解PH并做出正确诊断对于评估最合适的治疗策略至关重要。在本文中，已结合系统性硬化症的临床进展并聚焦于不同的潜在发病机制对PH的新分类标准进行了研究。
