Medical School, University of Cyprus, Nicosia CY2024, Cyprus.
Int J Mol Sci. 2023 Mar 7;24(6):5085. doi: 10.3390/ijms24065085.
Pulmonary Hypertension (PH) is a common manifestation in patients with Systemic Lupus Erythematosus (SLE) and varies from asymptomatic to life-threatening disease. PH can result not only from immune system dysregulation, but also from various conditions, including cardiorespiratory disorders and thromboembolic diseases. Most commonly, SLE-related PH presents with non-specific symptoms, such as progressive dyspnea on exertion, generalized fatigue and weakness and eventually dyspnea at rest. Prompt diagnosis of SLE-related PH and early identification of the underlying pathogenetic mechanisms is demanded in order to introduce targeted therapy to prevent irreversible pulmonary vascular damage. In most cases the management of PH in SLE patients is similar to idiopathic pulmonary arterial hypertension (PAH). Furthermore, specific diagnostic tools like biomarkers or screening protocols, to establish early diagnosis seem to be not available yet. Although, the survival rates for patients with SLE-related PH vary between studies, it is evident that PH presence negatively affects the survival of SLE patients.
肺动脉高压（PH）是红斑狼疮（SLE）患者的常见表现，其严重程度从无症状到危及生命不等。PH 不仅可能由免疫系统失调引起，还可能由多种情况引起，包括心肺疾病和血栓栓塞性疾病。最常见的是，SLE 相关 PH 表现为非特异性症状，如运动时进行性呼吸困难、全身疲劳和虚弱，最终发展为静息时呼吸困难。为了引入靶向治疗以防止不可逆的肺血管损伤，需要及时诊断 SLE 相关 PH 并早期识别潜在的发病机制。在大多数情况下，SLE 患者的 PH 管理与特发性肺动脉高压（PAH）相似。此外，目前似乎还没有建立早期诊断的特定诊断工具，如生物标志物或筛查方案。尽管 SLE 相关 PH 患者的生存率因研究而异，但 PH 的存在显然会影响 SLE 患者的生存率。
