Aberle Teresa, Bourn Rebecka L, Munroe Melissa E, Chen Hua, Roberts Virginia C, Guthridge Joel M, Bean Krista, Robertson Julie M, Sivils Kathy L, Rasmussen Astrid, Liles Meghan, Merrill Joan T, Harley John B, Olsen Nancy J, Karp David R, James Judith A
Oklahoma Medical Research Foundation, Oklahoma City.
Cincinnati Children's Hospital Medical Center and US Department of Veterans Affairs Medical Center, Cincinnati, Ohio.
Arthritis Care Res (Hoboken). 2017 Dec;69(12):1780-1788. doi: 10.1002/acr.23201. Epub 2017 Nov 14.
Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for systemic lupus erythematosus (SLE) classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined the clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients.
Medical records of subjects enrolled in the Lupus Family Registry and Repository were reviewed for medication history and American College of Rheumatology (ACR) classification criteria to identify ILE patients (3 ACR criteria; n = 440) and SLE patients (≥4 ACR criteria; n = 3,397). Participants completed the Connective Tissue Disease Screening Questionnaire. Anticardiolipin and plasma B lymphocyte stimulator (BLyS) were measured by enzyme-linked immunosorbent assay, antinuclear antibodies (ANAs) by indirect immunofluorescence, and 13 autoantibodies by bead-based assays.
On average, ILE patients were older than SLE patients (46.2 years versus 42.0 years; P < 0.0001), and fewer ILE patients were African American (23.9% versus 32.2%; P < 0.001). ILE patients exhibited fewer autoantibody specificities than SLE patients (1.3 versus 2.6; P < 0.0001) and were less likely to have ANA titers ≥1:1,080 (10.5% versus 19.5%; P < 0.0001). BLyS levels were intermediate in ILE patients (controls < ILE; P = 0.016; ILE < SLE; P = 0.008). Pericarditis, renal, or neurologic manifestations occurred in 12.5% of ILE patients and were associated with non-European American race/ethnicity (P = 0.012). Hydroxychloroquine use increased over time, but was less frequent in ILE than SLE patients (65.2% versus 83.1%; P < 0.0001).
Although usually characterized by milder symptoms, ILE manifestations may require immunomodulatory treatments. Longitudinal studies are necessary to understand how ILE affects organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.
不完全性红斑狼疮（ILE）涉及与系统性红斑狼疮（SLE）分类相符但不足以确诊的临床和/或血清学表现。由于对ILE的本质了解不足且尚无治疗建议，我们研究了不同的ILE和SLE患者群体的临床表现、用药史及免疫学特征。
回顾狼疮家族登记与资料库中受试者的病历，以获取用药史及美国风湿病学会（ACR）分类标准，从而确定ILE患者（3条ACR标准；n = 440）和SLE患者（≥4条ACR标准；n = 3397）。参与者完成结缔组织病筛查问卷。采用酶联免疫吸附测定法检测抗心磷脂和血浆B淋巴细胞刺激因子（BLyS），采用间接免疫荧光法检测抗核抗体（ANA），并采用基于微珠的检测法检测13种自身抗体。
ILE患者的平均年龄大于SLE患者（46.2岁对42.0岁；P < 0.0001），非裔美国ILE患者较少（23.9%对32.2%；P < 0.001）。ILE患者表现出的自身抗体特异性少于SLE患者（1.3种对2.6种；P < 0.0001），且ANA滴度≥1:1080的可能性较小（10.5%对19.5%；P < 0.0001）。ILE患者的BLyS水平处于中间值（对照组 < ILE；P = 0.016；ILE < SLE；P = 0.008）。12.5%的ILE患者出现心包炎、肾脏或神经系统表现，且与非欧美种族/族裔相关（P = 0.012）。羟氯喹的使用随时间增加，但ILE患者的使用频率低于SLE患者（65.2%对83.1%；P < 0.0001）。
尽管ILE通常表现为症状较轻，但可能需要免疫调节治疗。有必要进行纵向研究以了解ILE如何影响器官损害及未来发生SLE的风险，并明确ILE特有的分子途径。
