2021皮肤红斑狼疮的诊断、治疗和长期管理指南

Guideline for the Diagnosis,Treatment and Long-Term Management of Cutaneous Lupus Erythematosus

皮肤红斑狼疮(CLE)是一种炎症性自身免疫性疾病,其分型较多,包括急性、亚急性、慢性和间歇性CLE。其中,慢性CLE可以进一步分为红斑狼疮(LE)的几个亚型,如盘状LE、疣状LE、深在性LE、冻疮样LE和Blaschko线状LE等。为了给皮肤科医生和风湿科医生提供CLE的诊断、治疗和长期管理的实用指导建议,本证据和共识指南根据卫生保健实践指南的报告条目(国际RIGHT工作组)目录编制,并在国际实践指南注册平台注册。在亚洲皮肤科学会(ADA)、亚洲皮肤病性病学会(AADV)以及中华医学会皮肤性病学分会(CSD)红斑狼疮研究中心的共同努力下,来自亚洲、美洲、欧洲等16个国家或地区的25名皮肤科医生、7名风湿科医生、1名红斑狼疮领域专家和2名方法学家参与了本指南的制定。所有建议均取得了32名医生中至少80%的同意。作为共识,CLE的诊断主要基于临床和组织病理学表现的评估,并且通过对全身多系统损伤的评估,排除系统性红斑狼疮(SLE)。外用糖皮质激素和钙调磷酸酶抑制剂是局部CLE的一线治疗。对于泛发或严重的CLE皮损和/或局部耐药的病例,可以合并全身治疗,包括抗疟药和/或短期全身使用糖皮质激素。值得注意的是,抗疟药是所有类型CLE的一线全身治疗药物,同样可用于妊娠期患者和儿童。二线治疗用药包括沙利度胺、类维生素A类药物、氨苯砜和甲氨蝶呤,而霉酚酸酯是三线治疗用药。最后,脉冲染料激光或手术可作为四线治疗,用于局部以及发生在不可修饰部位的慢性、难治性CLE皮损。贝利尤单抗亦可作为四线治疗用药,用于活动性SLE患者的泛发性CLE皮损或糖皮质激素逐渐减量期间复发的急性CLE。对于疾病的管理,患者教育和长期随访是必要的。建议在每次随访时适当评估疾病活动度、皮肤和其他器官损伤、生活质量、合并症及可能发生的不良事件。

Cutaneous lupus erythematosus(CLE)is an infl ammatory,autoimmune disease encompassing a broad spectrum of subtypes including acute,subacute,chronic and intermittent CLE.Among these,chronic CLE can be further classifi ed into several subclasses of lupus erythematosus(LE)such as discoid LE,verrucous LE,LE profundus,chilblain LE and Blaschko linear LE.To provide all dermatologists and rheumatologists with a practical guideline for the diagnosis,treatment and long-term management of CLE,this evidence-and consensus-based guideline was developed following the checklist established by the international Reporting Items for Practice Guidelines in Healthcare(RIGHT)Working Group and was registered at the International Practice Guideline Registry Platform.With the joint efforts of the Asian Dermatological Association(ADA),the Asian Academy of Dermatology and Venereology(AADV)and the Lupus Erythematosus Research Center of Chinese Society of Dermatology(CSD),a total of 25 dermatologists,7 rheumatologists,one research scientist on lupus and 2 methodologists,from 16 countries/regions in Asia,America and Europe,participated in the development of this guideline.All recommendations were agreed on by at least 80%of the 32 voting physicians.As a consensus,diagnosis of CLE is mainly based on the evaluation of clinical and histopathological manifestations,with an exclusion of systemic lupus erythematosus(SLE)by assessment of systemic involvement.For localized CLE lesions,topical corticosteroids and topical calcineurin inhibitors are fi rst-line treatment.For widespread or severe CLE lesions and(or)cases resistant to topical treatment,systemic treatment including antimalarials and(or)short-term corticosteroids can be added.Notably,antimalarials are the fi rst-line systemic treatment for all types of CLE,and can also be used in pregnant patients and pediatric patients.Second-line choices include thalidomide,retinoids,dapsone and methotrexate,whereas mycophenolate mofetil,is third-line treatment.Finally,pulsed-dye laser or surgery can be added as fourth-line treatment for localized,refractory lesions of CCLE in cosmetically unacceptable areas,whereas belimumab may be used as fourth-line treatment for widespread CLE lesions in patients with active SLE,or recurrence of ACLE during tapering of corticosteroids.As for management of the disease,patient education and a long-term follow-up are necessary.Disease activity,damage of skin and other organs,quality of life,comorbidities and possible adverse events are suggested to be assessed in every follow-up visit,when appropriate.