Systemic Symptoms in the Progression of Cutaneous to Systemic Lupus Erythematosus
Systemic Symptoms in the Progression of Cutaneous to Systemic Lupus Erythematosus
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DOI：
10.1001/jamadermatol.2013.9026
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发表时间：
2014-03-01
期刊：
影响因子：
10.9
通讯作者：
Werth, Victoria P.
中科院分区：
文献类型：
--
作者：
Wieczorek, Isabela T.;Propert, Kathleen J.;Werth, Victoria P.
重要性皮肤红斑狼疮（CLE）患者发展为系统性红斑狼疮（SLE）可能有很少和轻微的全身症状。目的描述CLE患者纵向队列中全身症状的类型和严重程度。对2007年1月至2011年4月在大学自身免疫性皮肤病诊所就诊的77例CLE患者进行的纵向队列研究。结果77例CLE患者中，13例（17%）符合SLE诊断标准，从CLE诊断到SLE的平均（SD）时间为8.03（6.20）年。在13例患者中，1例（8%）仅符合美国风湿病学会（ACR）的颊皮疹、盘状皮疹、光敏性和口腔溃疡的粘膜皮肤标准，3例（23%）符合粘膜皮肤ACR标准加上阳性抗核抗体和其他抗体滴度。平均（SD）随访时间为2.81（1.34）年，13名进展到符合SLE标准的CLE患者中只有5名（38%）发展为中度至重度的其他全身性疾病。并且大多数在研究期间没有发展到轻度的额外的全身性疾病。因此，我们的研究表明，一小部分CLE患者最终发展为SLE，即使他们这样做，大多数患者将有轻微的全身性疾病。
IMPORTANCE Patients with cutaneous lupus erythematosus (CLE) who develop systemic lupus erythematosus (SLE) may have few and mild systemic symptoms.OBJECTIVE To characterize the types and severity of systemic symptoms in a longitudinal cohort of patients with CLE.DESIGN, SETTING, AND PARTICIPANTS Prospective, longitudinal cohort study of 77 patients with CLE who presented between January 2007 and April 2011 at a university autoimmune skin disease clinic.MAIN OUTCOMES AND MEASURES Systemic symptoms and severity were determined from data recorded at each study visit and from medical records.RESULTS Of 77 patients with CLE, 13 (17%) went on to meet criteria for SLE, with a mean (SD) time from CLE diagnosis to SLE of 8.03 (6.20) years. Of the 13 patients, 1 (8%) solely met the mucocutaneous American College of Rheumatology (ACR) criteria of malar rash, discoid rash, photosensitivity, and oral ulcers, and 3 (23%) met the mucocutaneous ACR criteria plus positive antinuclear and other antibody titers. After a mean (SD) follow-up time of 2.81 (1.34) years, only 5 of the 13 patients with CLE (38%) who progressed to meet SLE criteria developed moderate to severe additional systemic disease.CONCLUSIONS AND RELEVANCE Patients with CLE who developed SLE during our study did so mostly by meeting the mucocutaneous ACR criteria, and the majority developed none to mild additional systemic disease during the study period. Thus, our study suggests that a small percentage of patients with CLE eventually develop SLE and that even if they do, most patients will have mild systemic disease.