Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.
Duke-NUS Medical School, Singapore.
Lupus. 2023 Jul;32(8):952-963. doi: 10.1177/09612033231180704. Epub 2023 Jun 2.
Diffuse alveolar haemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). We describe the clinical characteristics, treatment and survival outcomes of SLE patients with DAH in Singapore.
We conducted a retrospective review of the medical records of SLE patients with DAH hospitalised in 3 tertiary hospitals between January 2007 and October 2017. Patient demographics, clinical characteristics, laboratory, radiologic and bronchoscopic findings, as well as the treatments, were compared between survivors and non-survivors. Survival rates were analysed between the various treatment groups.
A total of 35 patients with DAH were included in this study. Majority of them were female (71.4%) and of Chinese ethnicity (62.9%). Median age was 40.0 years (IQR: 25-54), with a median disease duration of 8.9 months (IQR: 0.13-102.4). Haemoptysis was the most common clinical presentation, and majority had concomitant cytopaenia and lupus nephritis. All patients received high dose glucocorticoids; 27 (77.1%), 16 (45.7%) and 23 (65.7%) received cyclophosphamide (CYP), rituximab (RTX), and plasmapheresis (PLEX), respectively. Twenty-two patients required mechanical ventilation with a median duration of 12 days. Overall mortality rate was 40%, with a median survival time of 162 days. Twenty-six patients (74.3%) achieved remission, with an overall median time to remission of 12 days (IQR: 6-46) after diagnosis of DAH. Patients on triple therapy (CYP, RTX and PLEX) had a median survival of 162 days as compared to 14 days in patients on PLEX alone ( = .0026).
The overall mortality of DAH in SLE patients remained high. There were no significant differences in patient demographics or clinical characteristics between the survivors and non-survivors. However, better survival appears to be associated with treatment with cyclophosphamide.
弥漫性肺泡出血（DAH）是系统性红斑狼疮（SLE）的一种罕见但危及生命的并发症。我们描述了在新加坡住院的 SLE 患者发生 DAH 的临床特征、治疗方法和生存结局。
我们对 2007 年 1 月至 2017 年 10 月期间在 3 家三级医院住院的 SLE 合并 DAH 患者的病历进行了回顾性分析。比较了幸存者和非幸存者之间的患者人口统计学、临床特征、实验室、影像学和支气管镜检查结果以及治疗方法。分析了不同治疗组之间的生存率。
本研究共纳入 35 例 DAH 患者，其中大多数为女性（71.4%）和华裔（62.9%）。中位年龄为 40.0 岁（IQR：25-54），中位病程为 8.9 个月（IQR：0.13-102.4）。咯血是最常见的临床表现，大多数患者同时伴有细胞减少症和狼疮肾炎。所有患者均接受了大剂量糖皮质激素治疗；27（77.1%）、16（45.7%）和 23（65.7%）例患者分别接受了环磷酰胺（CYP）、利妥昔单抗（RTX）和血浆置换（PLEX）治疗。22 例患者需要机械通气，中位时间为 12 天。总体死亡率为 40%，中位生存时间为 162 天。26 例（74.3%）患者达到缓解，从 DAH 诊断到缓解的中位时间为 12 天（IQR：6-46）。与单独接受 PLEX 治疗的患者（ =.0026）相比，接受 CYP、RTX 和 PLEX 三联治疗的患者中位生存时间为 162 天。
SLE 患者 DAH 的总体死亡率仍然较高。幸存者和非幸存者之间在患者人口统计学或临床特征方面无显著差异。然而，更好的生存似乎与环磷酰胺治疗有关。
