Calderon-Valverde Gabriel, Quiros-Meza Mariana, Alfaro-Murillo Alberto
Rheumatology, Universidad de Costa Rica - Hospital San Juan de Dios, San Jose, CRI.
Internal Medicine, Universidad de Costa Rica - Hospital San Juan de Dios, San Jose, CRI.
Cureus. 2024 Aug 18;16(8):e67148. doi: 10.7759/cureus.67148. eCollection 2024 Aug.
Systemic lupus erythematosus (SLE) is a multifaceted autoimmune disorder that presents with a wide array of clinical features, including autoimmune hemolysis and nephritis. Autoimmune hemolysis in SLE is typically linked to warm antibodies, but the occurrence of cold agglutinin syndrome (CAS), driven by cold-reactive antibodies, is exceptionally rare. Lupus nephritis (LN) is among the most severe complications of SLE, characterized by immune complex-mediated glomerulonephritis, which often leads to considerable morbidity and mortality. Nephritis in SLE is a major indicator of chronic kidney disease, with many patients experiencing progressive renal damage over time. Early diagnosis and individualized treatment approaches are crucial for effectively managing these intertwined conditions. This case report presents a distinct clinical scenario involving a 53-year-old Hispanic female diagnosed with SLE, who concurrently presented with CAS and nephritis. The patient's initial symptoms included chest pain, severe macrocytic anemia, elevated creatinine levels, and evidence of active hemolysis. CAS was diagnosed through a positive direct antiglobulin test for C3d and elevated cold agglutinin titers. Further comprehensive assessments revealed dysgammaglobulinemia, hypocomplementemia, and positive anti-Ro antibodies, with a renal biopsy confirming LN (ISN/RPS Class IV and Class V). The patient exhibited a favorable response to a treatment regimen comprising high-dose steroids and anti-CD20 therapy, resulting in the complete cessation of hemolysis and a >50% decrease in proteinuria after six months. This case underscores the rarity of CAS in the context of SLE, particularly when coupled with nephritis, and highlights the need for tailored treatment strategies. Anti-CD20 therapy, as used in primary CAS management, emerges as a promising option for this unique presentation, offering insights into the complex interplay of autoimmune conditions.
系统性红斑狼疮（SLE）是一种多方面的自身免疫性疾病，具有广泛的临床特征，包括自身免疫性溶血和肾炎。SLE中的自身免疫性溶血通常与温抗体有关，但由冷反应性抗体驱动的冷凝集素综合征（CAS）的发生极为罕见。狼疮性肾炎（LN）是SLE最严重的并发症之一，其特征是免疫复合物介导的肾小球肾炎，常导致相当高的发病率和死亡率。SLE中的肾炎是慢性肾脏病的主要指标，许多患者随着时间的推移会出现进行性肾损害。早期诊断和个体化治疗方法对于有效管理这些相互交织的病症至关重要。本病例报告呈现了一个独特的临床案例，涉及一名53岁的西班牙裔女性，被诊断为SLE，同时患有CAS和肾炎。患者的初始症状包括胸痛、严重的大细胞性贫血、肌酐水平升高以及活跃溶血的证据。通过C3d直接抗球蛋白试验阳性和冷凝集素滴度升高诊断为CAS。进一步的综合评估显示γ球蛋白异常、补体减少和抗Ro抗体阳性，肾活检证实为LN（国际肾脏病学会/肾脏病理学会分类IV级和V级）。患者对包括大剂量类固醇和抗CD20治疗的治疗方案表现出良好反应，六个月后溶血完全停止，蛋白尿减少>50%。本病例强调了在SLE背景下CAS的罕见性，特别是与肾炎同时存在时，并突出了制定个性化治疗策略的必要性。在原发性CAS管理中使用的抗CD20治疗，对于这种独特表现而言是一种有前景的选择，为自身免疫性疾病的复杂相互作用提供了见解。
