New Guidelines Include Updates for CLE Treatment, Management

In a recently published Journal of Autoimmunity article, researchers from across America, Asia, and Europe outlined an evidence- and consensus-based guideline to provide dermatologists and rheumatologists with practical recommendations for diagnosis, treatment, and long-term management of cutaneous lupus erythematosus (CLE).

The guidelines are based on a checklist established by the international Reporting Items for Practice Guidelines in Healthcare (RIGHT) Working Group. Specifically, the standards agreed upon by at least 80% of the 32 voting physicians include the statements that evaluation of systemic involvement is needed to exclude systemic lupus erythematosus (SLE) prior to diagnosing CLE and that patients with CLE require education and long-term follow-up.

“Whereas SLE generally involves multiple organs and systems, CLE mainly affects skin and mucosa and can be further classified into a couple of distinct subtypes based on their different clinical and histopathological features as well as serological findings,” authors explained.

However, due to the low incidence of many subtypes and the complex classification, correctly diagnosing CLE can be challenging. In addition, only 1 drug or therapeutic intervention—hydroxychloroquine—is licensed for CLE, and there are no standardized diagnostic criteria for each subtype.

“With clinical progress in recent years, increasingly accumulated new evidence and updated opinions need to be incorporated in the guideline,” researchers wrote.

The guideline also encompasses recommendations made in previous updates published in 2017 and 2019. In total, 25 dermatologists, 7 rheumatologists, 1 research scientist on lupus, and 2 methodologists from 16 countries helped develop this update.

When it comes to diagnosing the condition, in addition to ruling out an SLE diagnosis, experts recommend “an assessment of disease activity and damage of CLE at baseline before treatment and at each follow-up, when appropriate, during treatment.”

Physicians should also exclude other organ damage beyond skin lesions prior to establishing a diagnosis, as skin lesions of other lupus subtypes could present in patients with SLE.

The heterogeneity of the disease underscores the importance of individualized treatment, researchers added. Recommendations involving risk factors and protective measures for CLE include protection against ultraviolet (UV) and sunlight exposure, cessation of smoking, undertaking a thorough history of drug/medicine use, and avoidance of trauma, surgery, cryotherapy, and invasive laser treatment of the skin unless it is necessary or unavoidable or its benefit overweighs the risk among those with of a history of Koebner phenomenon.

Consensus-based treatment recommendations include:

• For localized CLE lesions, topical corticosteroids and topical calcineurin inhibitors are first-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.
• Antimalarials are the first-line systemic treatment for all types of CLE and can be used in pregnant patients and pediatric patients.

Second-line choices include thalidomide, retinoids, dapsone, and methotrexate (MTX), whereas mycophenolate mofetil (MMF) is third-line treatment.

Pulsed-dye laser or surgery can be added as fourth-line treatment for localized, refractory lesions of chronic CLE 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 acute CLE during tapering of corticosteroids.

As CLE is chronic and relapsing in nature, experts recommend that providers assess disease activity, skin and other organ damage, comorbidities, and possible adverse events during each follow-up visit when appropriate.

For patients looking to become pregnant, “we recommend a preconception evaluation on the control of lupus, other risk factors of pregnancy and medication use for patients with CLE preparing for pregnancy, preferentially by both an obstetrician and a dermatologist or rheumatologist,” they added.

This evaluation is necessary to help minimize the risk of both maternal and fetal complications. Regular, close monitoring of the disease during pregnancy and in the perinatal period can also help mitigate these risks.

Overall, “evaluation of systemic involvement is necessary to exclude the diagnosis of SLE and should be a crucial part of disease monitoring for patients with CLE during the follow-up period,” authors concluded. “As a basis of disease management for all patients with CLE, patient education and a long-term follow-up are always necessary to help monitor the disease activity, systemic involvement, comorbidities and possible adverse events.”

Phase III clinical trials are still warranted to better understand some of the recommended treatments’ efficacy, safety, and practical value in CLE subtypes.

Reference

Lu Q, Long H, Chow S, et al. Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus. J Autoimmun. 2021;123:102707. doi:10.1016/j.jaut.2021.102707