Hospices Civils de Lyon, Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Lyon, France.
Université Lyon I, Lyon, France.
Arthritis Res Ther. 2022 Jul 14;24(1):167. doi: 10.1186/s13075-022-02857-z.
The clinical value of cryoglobulinemia (CG) in systemic lupus erythematosus (SLE) is largely unknown. The aim of this retrospective study was to describe the characteristics of CG in SLE, its impact on SLE phenotype, and the features associated with cryoglobulinemic vasculitis (CryoVas) in SLE patients.
This retrospective study conducted in a French university hospital reviewed the data from 213 SLE patients having been screened for CG between January 2013 and December 2017. SLE patients positive for CG were compared to SLE patients without CG. Patients were classified as CryoVas using the criteria of De Vita et al. RESULTS: Of the 213 SLE patients included (mean age 29.2 years, female sex 85%), 142 (66%) had at least one positive CG in their history, 67% of them having a persistent CG at follow-up. CG was type III in 114 (80%) cases and type II in 27 (19%) cases. The mean concentration of the cryoprecipitate was 40mg/L (range 0-228). Patients with CG had significantly more C4 consumption. Among patients with CG, 21 (15%) developed a CryoVas. The clinical manifestations of patients with CryoVas were mainly cutaneous (purpura, ulcers, digital ischemia) and articular, without any death at follow-up. Severe manifestations of CG included glomerulonephritis in 1/21 (5%) patients and central nervous system involvement in 4/21 (19%) patients. A response to first-line treatments was observed in 12/13 (92%) patients, but relapses were observed for 3 of them.
CG is frequent in SLE, but mostly asymptomatic. CryoVas features involve mostly joints, skin, and general symptoms. CryoVas in SLE appears to be a specific condition, with a low prevalence of neuropathy, membranoproliferative glomerulonephritis, and severe manifestations.
冷球蛋白血症（Cryoglobulinemia，CG）在系统性红斑狼疮（Systemic Lupus Erythematosus，SLE）中的临床价值尚不清楚。本回顾性研究旨在描述SLE 患者 CG 的特征、对SLE 表型的影响以及与 SLE 患者冷球蛋白血管炎（Cryoglobulinemic Vasculitis，CryoVas）相关的特征。
这项在法国一所大学医院进行的回顾性研究，对 2013 年 1 月至 2017 年 12 月期间筛查 CG 的 213 例 SLE 患者的数据进行了回顾。将 CG 阳性的 SLE 患者与 CG 阴性的 SLE 患者进行比较。患者根据 De Vita 等人的标准分为 CryoVas 组。
213 例 SLE 患者中，142 例（平均年龄 29.2 岁，女性 85%）至少有一次 CG 阳性，67%的患者随访时有持续性 CG。CG 为 III 型 114 例（80%），II 型 27 例（19%）。冷沉淀物的平均浓度为 40mg/L（范围 0-228）。CG 患者的 C4 消耗明显更多。在 CG 患者中，21 例（15%）发生了 CryoVas。CryoVas 患者的临床表现主要为皮肤（紫癜、溃疡、手指缺血）和关节，随访中无死亡病例。CG 的严重表现包括 1/21（5%）患者的肾小球肾炎和 4/21（19%）患者的中枢神经系统受累。13 例患者（92%）对一线治疗有反应，但其中 3 例复发。
CG 在 SLE 中很常见，但大多无症状。CryoVas 的特征主要涉及关节、皮肤和全身症状。SLE 中的 CryoVas 似乎是一种特定的疾病，神经病变、膜性增殖性肾小球肾炎和严重表现的患病率较低。
