Abstract：
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.
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.
摘要：
冷球蛋白血症（CG）在系统性红斑狼疮（SLE）中的临床价值尚不清楚。这项回顾性研究的目的是描述SLE中CG的特征，它对SLE表型的影响，以及与SLE患者的冷球蛋白性血管炎（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月期间对213名SLE患者进行CG筛查的数据。将CG阳性的SLE患者与无CG的SLE患者进行比较。使用DeVita等人的标准将患者分类为CryoVas。结果：纳入213例SLE患者（平均年龄29.2岁，女性85%)，142（66％）在他们的历史中至少有一个CG阳性，67%的患者在随访时具有持续性CG。CG在114例（80％）中为III型，在27例（19％）中为II型。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(15%)发展了CryoVas。CryoVas患者的临床表现主要是皮肤（紫癜，溃疡,数字缺血)和关节，在后续行动中没有任何死亡。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例观察到复发。
CG在SLE中频繁出现，但大多是无症状的.CryoVas特征主要涉及关节，皮肤,和一般症状。SLE中的CryoVas似乎是一种特殊的情况，神经病的患病率较低，膜增生性肾小球肾炎，和严重的表现。
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月期间对213名SLE患者进行CG筛查的数据。将CG阳性的SLE患者与无CG的SLE患者进行比较。使用DeVita等人的标准将患者分类为CryoVas。结果：纳入213例SLE患者（平均年龄29.2岁，女性85%)，142（66％）在他们的历史中至少有一个CG阳性，67%的患者在随访时具有持续性CG。CG在114例（80％）中为III型，在27例（19％）中为II型。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(15%)发展了CryoVas。CryoVas患者的临床表现主要是皮肤（紫癜，溃疡,数字缺血)和关节，在后续行动中没有任何死亡。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例观察到复发。
CG在SLE中频繁出现，但大多是无症状的.CryoVas特征主要涉及关节，皮肤,和一般症状。SLE中的CryoVas似乎是一种特殊的情况，神经病的患病率较低，膜增生性肾小球肾炎，和严重的表现。
