Pulmonary hypertension (PH) is a known complication of certain connective tissue diseases (CTDs), with systemic sclerosis (SSc) being the most common in the Western world. However, PH in association with non-SSc CTD such as systemic lupus erythematous, mixed connective tissue disease, and primary Sjögren's syndrome constitutes a distinct subset of patients with inherently different epidemiologic profiles, pathophysiologic mechanisms, clinical features, therapeutic options, and prognostic implications. The purpose of this review is to inform a practical approach for clinicians evaluating patients with non-SSc CTD-associated PH.

The development of PH in these patients involves a complex interplay between genetic factors, immune-mediated mechanisms, and endothelial cell dysfunction. Furthermore, the broad spectrum of CTD manifestations can contribute to the development of PH through various pathophysiologic mechanisms, including intrinsic pulmonary arteriolar vasculopathy (pulmonary arterial hypertension, Group 1 PH), left-heart disease (Group 2), chronic lung disease (Group 3), chronic pulmonary artery obstruction (Group 4), and unclear and/or multifactorial mechanisms (Group 5). The importance of diagnosing PH early in symptomatic patients with non-SSc CTD is highlighted, with a review of the relevant biomarkers, imaging, and diagnostic procedures required to establish a diagnosis.

Therapeutic strategies for non-SSc PH associated with CTD are explored with an in-depth review of the medical, interventional, and surgical options available to these patients, emphasizing the CTD-specific considerations that guide treatment and aid in prognosis. By identifying gaps in the current literature, we offer insights into future research priorities that may prove valuable for patients with PH associated with non-SSc CTD.

肺动脉高压（PH）是某些结缔组织疾病（CTD）的一种已知并发症，其中系统性硬化症（SSc）在西方世界最常见。然而，PH 与非 SSc CTD（如系统性红斑狼疮、混合结缔组织病和原发性干燥综合征）相关，构成了一个独特的患者亚群，具有本质上不同的流行病学特征、病理生理学机制、临床特征、治疗选择和预后影响。本综述的目的是为临床医生提供评估非 SSc CTD 相关 PH 患者的实用方法。

这些患者 PH 的发生涉及遗传因素、免疫介导机制和内皮细胞功能障碍之间复杂的相互作用。此外，广泛的 CTD 表现可通过各种病理生理机制促进 PH 的发展，包括内源性肺小动脉血管病变（肺动脉高压，第 1 组 PH）、左心疾病（第 2 组）、慢性肺病（第 3 组） ）、慢性肺动脉阻塞（第 4 组）以及不清楚和/或多因素机制（第 5 组）。强调了在有症状的非 SSc CTD 患者中早期诊断 PH 的重要性，并对建立诊断所需的相关生物标志物、影像学和诊断程序进行了回顾。

通过深入审查这些患者可用的医疗、介入和手术选择，探讨了与 CTD 相关的非 SSc PH 的治疗策略，强调指导治疗和帮助预后的 CTD 特异性考虑因素。通过确定当前文献中的空白，我们提供了对未来研究重点的见解，这对于与非 SSc CTD 相关的 PH 患者可能有价值。