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. 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. • Cryoglobulinemia is frequent in SLE, but mostly asymptomatic. • Sixty-six percent of SLE patients tested positive for cryoglobulins, and 15% of the SLE patients with cryoglobulinemia developed a cryoglobulinemic vasculitis. • Features of the cryoglobulinemic vasculitis mainly involved skin, joints, and general signs. Severe manifestations of vasculitis were rare.

中文翻译：

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系统性红斑狼疮中的冷球蛋白血症：213 例患者的回顾性研究

系统性红斑狼疮 (SLE) 中冷球蛋白血症 (CG) 的临床价值在很大程度上是未知的。这项回顾性研究的目的是描述 SLE 中 CG 的特征、其对 SLE 表型的影响以及与 SLE 患者冷球蛋白血症性血管炎 (CryoVas) 相关的特征。这项在法国大学医院进行的回顾性研究回顾了 2013 年 1 月至 2017 年 12 月期间接受 CG 筛查的 213 名 SLE 患者的数据。将 CG 阳性的 SLE 患者与没有 CG 的 SLE 患者进行了比较。使用 De Vita 等人的标准将患者分类为 CryoVas。在纳入的 213 名 SLE 患者中（平均年龄 29.2 岁，女性 85%），142 名（66%）在他们的病史中至少有 1 次 CG 阳性，其中 67% 在随访时有持续 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 消耗量显着增加。在 CG 患者中，21 名（15%）发生了 CryoVas。CryoVas患者临床表现主要为皮肤（紫癜、溃疡、指端缺血）和关节，随访无死亡。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。在 12/13 (92%) 患者中观察到对一线治疗的反应，但其中 3 人观察到复发。CG 在 SLE 中很常见，但大多无症状。CryoVas 特征主要涉及关节、皮肤和一般症状。SLE 中的 CryoVas 似乎是一种特殊情况，神经病变、膜增生性肾小球肾炎和严重表现的患病率较低。• 冷球蛋白血症在 SLE 中很常见，但大多无症状。• 66% 的 SLE 患者冷球蛋白检测呈阳性，15% 的 SLE 患有冷球蛋白血症的患者发展为冷球蛋白血症性血管炎。• 冷球蛋白血症性血管炎的特征主要涉及皮肤、关节和全身体征。血管炎的严重表现很少见。和一般标志。血管炎的严重表现很少见。和一般标志。血管炎的严重表现很少见。