Abstract
Cutaneous lupus erythematosus (CLE) is an autoimmune disease that can be associated with systemic lupus erythematosus (SLE) symptoms. The pathogenesis of both CLE and SLE is multifactorial, involving genetic susceptibility, environmental factors, and innate and adaptive immune responses. Despite the efficacy of current medications, many patients remain refractory, highlighting the necessity for new treatment options. Unfortunately, owing to challenges related in part to trial design and disease heterogeneity, only one new biologic in the last 50 years has been approved by the US Food and Drug Administration for the treatment of SLE. Thus, although SLE and CLE have a similar pathogenesis, patients with CLE who do not meet criteria for SLE cannot benefit from this advancement. This article discusses the recent trials and emphasizes the need to include patients with single-organ lupus, such as CLE, in SLE trials.
Highlights
Lupus erythematosus (LE) is an autoimmune disease associated with a broad range of cutaneous LE (CLE) and systemic LE (SLE) symptoms[1]
CLE is divided into three primary subsets: acute CLE, subacute CLE (SCLE), and chronic CLE6
Chronic CLE is subsequently categorized as discoid LE (DLE), hypertrophic LE, LE profundus, chilblain CLE, and lupus tumidus[6]
Summary
Lupus erythematosus (LE) is an autoimmune disease associated with a broad range of cutaneous LE (CLE) and systemic LE (SLE) symptoms[1]. The antibody, when bound, leads to internalization of BDCA2, a pDC-specific receptor, and inhibits the production of type I IFNs and other inflammatory mediators[44] This phase Ib randomized, double-blind, placebo-controlled, multicenter clinical trial has confirmed the role of pDCs in SLE but has shown a decrease in cutaneous disease activity in these patients as compared with placebo[44]. Similar to thalidomide and its analogs, CC-220 is a high-affinity ligand for cereblon with immunomodulatory properties; its administration decreases Ikaros and Aiolos, two transcription factors encoded by their respective susceptibility loci, IKZF1 and IKZF3, which are associated with SLE45 This randomized, double-blind, placebo-controlled, phase IIa dose escalation study showed a strong correlation between improvement in CLASI score and pDC reduction[45]. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript
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