Department of Rheumatology and Immunology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, China.
Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.
Clin Rheumatol. 2024 Dec;43(12):3713-3722. doi: 10.1007/s10067-024-07216-8. Epub 2024 Nov 1.
Pulmonary hypertension (PH) is a severe complication of systemic lupus erythematosus (SLE). This study investigated the clinical features of patients with SLE-PH in China based on disease manifestations and organ involvement to define precise treatment of SLE and early prevention of complications.
In total, 205 SLE-PH patients were included in this study. A cluster analysis was applied according to six clinical and serological variables to define different subgroups of patients. The survival rates of SLE-PH patients and risk factors that influenced prognosis were also compared and a clinical prediction model was developed for the diagnosis of associated lupus nephritis (LN).
Patients were clustered in five groups. Patients in cluster 1 had severe renal damage (all patients had LN and had the highest creatinine and urea nitrogen and the lowest eGFR levels). Patients in cluster 2 had mild renal damage (more than half of the patients had associated LN and 87.5% had increased urinary protein levels but presented a lower degree of renal damage than those in the first group. Patients in cluster 3 had low-grade proteinuria (all patients had 24h urinary protein < 0.5g). Patients in cluster 4 had hematuria or urinary tubular damage (26% of patients had associated LN, but none of the patients had proteinuria. In cluster 5, patients barely had any major organ involvement. The clinical prediction model for a diagnosis of SLE-PH-LN was anti-dsDNA antibodies > 364.64 IU/mL and neutrophil-to-leukocyte ratio (NLR) > 5.55.
Our findings provide evidence indicating that SLE-PH presents varying clinical phenotypes and the treatment varies accordingly, suggesting the need for individualized treatment. Key Points • Clustered grouping of patients with SLE-PH is associated with renal injury. • This may be because PH and LN share a common pathogenesis. • The clinical prediction model for a diagnosis of SLE-PH-LN was anti-dsDNA antibodies >364.64 IU/mL and NLR >5.55.
肺动脉高压（PH）是系统性红斑狼疮（SLE）的严重并发症。本研究基于疾病表现和器官受累，对中国 SLE-PH 患者的临床特征进行调查，以明确 SLE 的治疗方法和早期并发症预防。
共纳入 205 例 SLE-PH 患者。应用聚类分析根据 6 个临床和血清学变量将患者分为不同亚组。比较 SLE-PH 患者的生存率和影响预后的危险因素，并建立诊断狼疮肾炎（LN）相关的临床预测模型。
患者分为 5 组。第 1 组患者有严重的肾脏损伤（所有患者均有 LN，且肌酐和尿素氮最高，eGFR 最低）。第 2 组患者有轻度肾脏损伤（超过一半的患者有相关 LN，87.5%的患者蛋白尿水平升高，但肾脏损伤程度低于第 1 组）。第 3 组患者有轻度蛋白尿（所有患者 24 小时尿蛋白<0.5g）。第 4 组患者有血尿或尿小管损伤（26%的患者有相关 LN，但无蛋白尿。第 5 组患者几乎没有任何主要器官受累。SLE-PH-LN 的临床预测模型为抗 dsDNA 抗体>364.64IU/mL 和中性粒细胞与白细胞比值（NLR）>5.55。
本研究结果表明，SLE-PH 表现出不同的临床表型，治疗方法也有所不同，提示需要个体化治疗。
•SLE-PH 患者聚类分组与肾脏损伤相关。
•这可能是因为 PH 和 LN 具有共同的发病机制。
•SLE-PH-LN 的临床预测模型为抗 dsDNA 抗体>364.64IU/mL 和 NLR>5.55。
