系統紅斑狼瘡併發肺高血壓症－－病例報告

Systemic Lupus Erythematosus with Pulmonary Hypertension

作者報告兩例全身性紅斑狼瘡併肺高血壓的病例。患者為34歲及26歲女性，診斷為全身性紅斑狼瘡的時間皆未超過三年。兩位病人皆在疾病進行過程中陸續產生肺高血壓。前者的雷諾氏現象較明顯且有腎臟病變，其肺高血壓之產生最可能是因為肺血管本身病變造成的血管動力異常。後者無腎臟之侵犯。其肺實質的換氣功能有缺陷，導致缺氧現象，此缺氧現象很可能即係其肺高血壓之原因。兩例的診斷皆靠脈動都卜勒超音波心圖。雖經血管擴張劑、類固醇及免疫抑制劑之治療，二位患者仍於呼吸困難症狀出現之後六個月內死亡。

The combination of SLE and pulmonary hypertension is quite uncommon. Only 46 patients have been reported since 1952, The treatment response of this setting is disappointing. We report two cases of SLE with pulmonary hypertension. The diagnoses were made by pulsed Doppler echocardiography which is a non- invasive instrument suitable for critical patients such as ours who could not undergo cardiac catheterization. The possible pathogenesis of these two cases is also briefly discussed.