National Pulmonary Hypertension Service, Royal Free Hospital NHS Foundation Trust, UK.
Best Pract Res Clin Rheumatol. 2013 Jun;27(3):425-34. doi: 10.1016/j.berh.2013.07.011.
Systemic lupus erythematosus is associated with several forms of pulmonary hypertension. It can cause pulmonary hypertension through pulmonary thromboembolic disease, left heart disease and lung disease as well as causing an isolated pulmonary vasculopathy called pulmonary arterial hypertension. The true prevalence of pulmonary arterial hypertension in patients with lupus is not known but probably is no more than 1%. Currently, treatment for lupus-associated pulmonary arterial hypertension is with pulmonary vasodilators including phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin analogues, as it is for other causes of pulmonary arterial hypertension. Case series suggest there may be a special role for immunosuppression in this rare group of patients. We present two brief case histories and summarise our experience over 15 years. Prognosis is better in lupus-associated pulmonary arterial hypertension than in systemic sclerosis-associated pulmonary arterial hypertension, but unfortunately it remains a fatal condition in most patients.
系统性红斑狼疮与多种形式的肺动脉高压有关。它可以通过肺血栓栓塞性疾病、左心疾病和肺部疾病引起肺动脉高压，并导致一种称为肺动脉高压的孤立性肺血管病变。狼疮患者中肺动脉高压的真正患病率尚不清楚，但可能不超过 1%。目前，治疗狼疮相关性肺动脉高压的方法是使用肺血管扩张剂，包括磷酸二酯酶-5 抑制剂、内皮素受体拮抗剂和前列环素类似物，与其他原因引起的肺动脉高压相同。病例系列研究表明，免疫抑制在这组罕见患者中可能具有特殊作用。我们提供了两个简短的病例报告，并总结了我们 15 年的经验。狼疮相关性肺动脉高压的预后优于系统性硬化症相关性肺动脉高压，但不幸的是，在大多数患者中，它仍然是一种致命的疾病。
