系统性红斑狼疮中单克隆丙种球蛋白血症与独特的临床病程、恶性肿瘤和死亡率相关：一项单中心回顾性队列研究。
Monoclonal gammopathy in systemic lupus erythematosus is associated with distinctive clinical course, malignancy and mortality rate: a single-centre retrospective cohort study.
机构信息
2nd Department of Internal Medicine, Jagiellonian University Medical College, Krakow, Poland.
Rheumatology and Immunology Clinical Department, University Hospital, Krakow, Poland.
出版信息
Lupus Sci Med. 2024 Aug 30;11(2):e001248. doi: 10.1136/lupus-2024-001248.
OBJECTIVES
Rheumatic diseases were previously associated with increased incidence of monoclonal gammopathy (MG) and its malignant transformation. The present study aimed to investigate the prevalence, malignant transformation risk, clinical correlates and prognostic impact of MG in SLE.
METHODS
A retrospective cohort study based on the medical records of n=1039 patients with SLE fulfilling the 1997 American College of Rheumatology (ACR), the 2019 European Alliance of Associations for Rheumatology (EULAR)/ACR and/or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria managed at two tertiary care departments of the University Hospital (Krakow, Poland) from January 2012 until November 2019.
RESULTS
SLE+MG cases were older at SLE diagnosis compared with non-MG SLE controls (53±15 years vs 37±15 years, respectively, p<0.01), had higher rates of lymphopenia, anaemia, haemolysis, serous effusions and interstitial lung disease (all p<0.05), and were more frequently treated with cyclophosphamide (57% vs 28%, p<0.01) or rituximab (13% vs 3%, p<0.01). Most MG cases were detected within a year after SLE diagnosis (Q25, Q75: 0, 12 years). With the median follow-up of 11 years (Q25, Q75: 6, 19 years), 34.8% (8 cases) of the SLE+MG cohort were diagnosed with malignancy, compared with 8.1% (82 cases) among the SLE controls (p<0.001). MG was associated with the relative hazard of death of HR 2.99 (95% CI 1.26 to 7.06, p<0.05) and a median survival time from SLE diagnosis to death of 5 years (Q25, Q75: 1, 14; range 0-41) for SLE+MG cases, as compared with 12 years (Q25, Q75: 6, 19; range 0-62) for the controls. The effect was non-independent on antimalarial medication use.
CONCLUSIONS
Our study emphasises heightened malignancy and mortality rates in SLE+MG cases. The association between immunosuppression, MG incidence and progression warrants further research.
目的
风湿性疾病以前与单克隆丙种球蛋白病（MG）及其恶性转化的发病率增加有关。本研究旨在探讨 MG 在系统性红斑狼疮（SLE）中的患病率、恶性转化风险、临床相关性和预后影响。
方法
本研究是一项基于病历的回顾性队列研究，共纳入了 1039 名符合 1997 年美国风湿病学会（ACR）、2019 年欧洲抗风湿病联盟（EULAR）/ACR 和/或 2012 年系统性红斑狼疮国际合作临床中心（SLICC）标准的 SLE 患者，这些患者于 2012 年 1 月至 2019 年 11 月在波兰克拉科夫大学医院的两个三级护理部门接受治疗。
结果
与非 MG SLE 对照组相比，SLE+MG 患者在 SLE 诊断时年龄更大（53±15 岁 vs 37±15 岁，p<0.01），淋巴细胞减少症、贫血、溶血性贫血、浆膜腔积液和间质性肺病的发生率更高（均 p<0.05），且更常接受环磷酰胺（57% vs 28%，p<0.01）或利妥昔单抗（13% vs 3%，p<0.01）治疗。大多数 MG 病例在 SLE 诊断后 1 年内被发现（Q25，Q75：0，12 年）。中位随访 11 年（Q25，Q75：6，19 年）后，SLE+MG 队列中有 34.8%（8 例）被诊断为恶性肿瘤，而 SLE 对照组中有 8.1%（82 例）（p<0.001）。MG 与死亡的相对危险度 HR 2.99（95%CI 1.26 至 7.06，p<0.05）和从 SLE 诊断到死亡的中位生存时间相关，SLE+MG 患者为 5 年（Q25，Q75：1，14；范围 0-41），而对照组为 12 年（Q25，Q75：6，19；范围 0-62）。该效果与抗疟药物的使用无关。
结论
我们的研究强调了 SLE+MG 患者恶性肿瘤和死亡率的升高。免疫抑制、MG 发生率和进展之间的关系需要进一步研究。
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