Huang C, Zhang S, Tian Z, Li M, Zeng X
Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
Department of Cardiology, Peking Union Medical College Hospital, Beijing China.
Lupus. 2014 Dec;23(14):1533-6. doi: 10.1177/0961203314552461. Epub 2014 Sep 26.
A 35-year-old female patient was diagnosed with pulmonary arterial hypertension (PAH) by right heart catheterization (RHC) in the 3rd month of systemic lupus erythematosus (SLE) duration with high disease activity. Through methylprednisolone pulse therapy, she got total recovery from PAH proven by repeat catheterization and follow-ups. This is the first simultaneously diagnosed, totally recovered, gold standard-proved SLE-PAH case ever reported in the literature. We would like to share this successful case, and at the same time, we want to highlight the relationship between presentation of PAH and active disease manifestations of SLE patients, and to discuss how immune suppressive treatment could benefit this subset of patients.
一名35岁女性患者在系统性红斑狼疮（SLE）病程第3个月、疾病活动度高时，经右心导管检查（RHC）诊断为肺动脉高压（PAH）。通过甲泼尼龙冲击治疗，经重复导管检查和随访证实她的PAH完全康复。这是文献中首次报道的同时诊断、完全康复且经金标准证实的SLE-PAH病例。我们希望分享这个成功案例，同时强调PAH的表现与SLE患者疾病活动表现之间的关系，并讨论免疫抑制治疗如何使这部分患者受益。
