Cutaneous Lupus Erythematosus: Current Treatment Options

Current Treatment Options in Rheumatology, Feb 2016

Opinion statement Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus (SLE). Managing CLE is important in order to reduce potential and established areas of damage, as well as improve quality of life (QOL). Non-drug therapy should be used in every case, which includes photoprotection, smoking cessation, and avoiding drugs that can trigger or exacerbate the disease. Topical corticosteroids and topical calcineurin inhibitors are often used in addition to lifestyle changes. Antimalarials are first-line systemic therapies, with hydroxychloroquine being the drug of choice. Quinacrine can be added to hydroxychloroquine for greater efficacy in hydroxychloroquine-refractory patients, and chloroquine can be used in place of hydroxychloroquine in patients who are unable to tolerate hydroxychloroquine. Second-line therapies include oral retinoids, immunosuppressives, immunomodulators, biologics, and pulsed dye laser. Systemic steroids may be necessary when bridging therapies but should normally be avoided due to their side effects. There is a paucity of high quality evidence with regard to management of cutaneous lupus, making it challenging to determine an appropriate treatment in refractory cases. Trials on existing therapies as well as new therapies are necessary in order to better treat patients with CLE.

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Cutaneous Lupus Erythematosus: Current Treatment Options

Curr Treat Options in Rheum Cutaneous Lupus Erythematosus: Current Treatment Options Jenna K. Presto Victoria P. Werth 0 0 Corporal Michael J. Crescenz Veterans Affairs Medical Center (Philadelphia) , Philadelphia, PA , USA Cutaneous lupus erythematosus I Topical steroids I Topical calcineurin inhibitors I Antimalarials I; Immunosuppressives I Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI™) - Opinion statement Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus (SLE). Managing CLE is important in order to reduce potential and established areas of damage, as well as improve quality of life (QOL). Non-drug therapy should be used in every case, which includes photoprotection, smoking cessation, and avoiding drugs that can trigger or exacerbate the disease. Topical corticosteroids and topical calcineurin inhibitors are often used in addition to lifestyle changes. Antimalarials are first-line systemic therapies, with hydroxychloroquine being the drug of choice. Quinacrine can be added to hydroxychloroquine for greater efficacy in hydroxychloroquine-refractory patients, and chloroquine can be used in place of hydroxychloroquine in patients who are unable to tolerate hydroxychloroquine. Second-line therapies include oral retinoids, immunosuppressives, immunomodulators, biologics, and pulsed dye laser. Systemic steroids may be necessary when bridging therapies but should normally be avoided due to their side effects. There is a paucity of high quality evidence with regard to management of cutaneous lupus, making it challenging to determine an appropriate treatment in refractory cases. Trials on existing therapies as well as new therapies are necessary in order to better treat patients with CLE. Introduction Lupus erythematosus (LE) is a broad-spectrum disease which can manifest as cutaneous lupus erythematosus (CLE) or multisystem organ disease with systemic lupus erythematosus (SLE). CLE is divided into subgroups, Cutaneous Lupus Erythematosus: Current Treatment Options consisting of chronic cutaneous lupus erythematosus (CCLE), subacute cutaneous lupus erythematosus (SCLE), and acute lupus erythematosus (ACLE). CCLE can be further divided; discoid lupus erythematosus (DLE) and lupus erythematosus tumidus (LET) are the most common forms of CCLE, while lupus panniculitis, hypertrophic lupus, mucosal lupus, chilblain lupus, and lichenoid DLE are less common forms of CCLE. The goal of cutaneous lupus treatment is to reduce potential and established areas of activity and damage. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI™) is a scoring system that has been validated to assess for skin disease activity and damage in cutaneous lupus patients. It has demonstrated intrarater and interrater reliability in dermatologists and rheumatologists [1, 2]. This scoring system has been used in numerous trials and has been shown to correlate with quality of life measures and disease improvement with a 4-point or 20 % decrease in CLASI™ activity score deemed clinically significant [2, 3]. Improving quality of life is an important factor in treatment. Quality of life in cutaneous lupus patients has been assessed using the Skindex 29 + 3 [4], a Lifestyle validated skin-specific quality of life instrument, and the SF-36 [5], a validated general health quality of life form [6]. Patients with cutaneous lupus have a worse quality of life (QOL) as compared to other dermatologic conditions, such as acne, non-melanoma skin cancer, and alopecia. Poor quality of life for CLE patients has been associated with female gender, generalized disease, severe disease, facial lesions, and younger age [7]. These QOL indicators have been found to be similar between geographic populations [8]. Antimalarial or antimetabolite treatment response leads to decreased emotion, functioning, and symptom score using the Skindex 29 [9 ]. This suggests the impact of treatment on a patient’s well-being. Treatment of cutaneous lupus should be based on severity and type of cutaneous lupus. Prevention of new lesions is the first key step. Prevention includes photoprotection, smoking cessation, and removal of exacerbating drugs. Topical therapy is often initiated with these preventative measures. Antimalarials remain the first-line systemic therapy and other systemic agents are used for recalcitrant cases or in patients who are unable to tolerate the antimalarials. UVA and UVB irradiation has been shown to induce lesions in patients with cutaneous lupus [ 10 ]. It is suggested that UV protection can reduce UV-triggered CLE lesions by inhibiting an interferon-mediated inflammatory response. CD11c- and CD123-positive dendritic cells, a source of interferons (IFNs), as well as IFN-induced human protein MxA are increased in unprotected irradiated skin of CLE patients and decreased when protected by broad spectrum sunscreen [ (...truncated)


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Jenna K. Presto, Victoria P. Werth. Cutaneous Lupus Erythematosus: Current Treatment Options, Current Treatment Options in Rheumatology, 2016, pp. 36-48, Volume 2, Issue 1, DOI: 10.1007/s40674-016-0033-z