Corporal Michael J. Crescenz VAMC and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania.
Arthritis Rheumatol. 2020 Nov;72(11):1777-1785. doi: 10.1002/art.41480. Epub 2020 Sep 30.
Cutaneous lupus erythematosus (CLE) is a chronic autoimmune disease that can present with a variety of skin manifestations and have a dramatic effect on a patient's quality of life. Effective treatment options for this disease are limited, and the efficacy of these treatments is often supported by low levels of evidence. This makes the treatment of refractory disease especially challenging, as it is difficult to achieve a consensus on the appropriate progression of treatment beyond first- and second-line treatment options. The treatment of refractory CLE often involves some degree of immunosuppression, which carries some risk for patients and requires a thoughtful approach to the selection of medications. Some treatments that have proven to be effective in systemic disease may not be as effective in cutaneous disease, making it difficult to extrapolate from the available evidence on systemic lupus erythematosus (SLE). Ultimately, the increased use of objective skin measurements in SLE clinical trials is necessary to understand drug efficacy in CLE and develop new treatments for this challenging disease. Here, we provide clinical examples of the challenges involved in treating refractory CLE, examine the evidence currently available for treatment options, and provide an algorithmic approach to the treatment of refractory disease based on this evidence. Novel therapies under development for CLE are also discussed, as they may soon be part of the accepted treatment regimen for refractory CLE.
盘状红斑狼疮（CLE）是一种慢性自身免疫性疾病，可表现为多种皮肤表现，对患者的生活质量产生重大影响。这种疾病的有效治疗选择有限，这些治疗的疗效往往得到低水平证据的支持。这使得难治性疾病的治疗特别具有挑战性，因为很难在一线和二线治疗方案之外就适当的治疗进展达成共识。难治性 CLE 的治疗通常涉及一定程度的免疫抑制，这对患者存在一定风险，需要慎重选择药物。一些已被证明对系统性疾病有效的治疗方法在皮肤疾病中可能效果不佳，因此很难从系统性红斑狼疮 (SLE) 的现有证据中推断出来。最终，有必要在 SLE 临床试验中更多地使用客观皮肤测量来了解 CLE 中的药物疗效，并为这种具有挑战性的疾病开发新的治疗方法。在这里，我们提供了治疗难治性 CLE 所涉及的临床实例，检查了目前可用于治疗选择的证据，并根据这些证据提供了治疗难治性疾病的算法方法。还讨论了正在开发用于 CLE 的新型疗法，因为它们可能很快成为难治性 CLE 公认的治疗方案的一部分。
