Thalidomide and Lenalidomide for Refractory Systemic/Cutaneous Lupus Erythematosus Treatment

A Narrative Review of Literature for Clinical Practice

Emily Figueiredo Neves Yuki, MD; Clovis A. Silva, MD, PhD; Nadia E. Aikawa, MD, PhD; Ricardo Romiti, MD, PhD; Carlos Otto Heise, MD, PhD; Eloisa Bonfa, MD, PhD; Sandra Gofinet Pasoto, MD, PhD


J Clin Rheumatol. 2021;27(6):248-259. 

In This Article

Assessment of SLE/CLE Skin Activity/Damage

In 2005, the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) was proposed by Albrecht et al., because of the difficulty of finding a validated instrument for assessing the clinical response of cutaneous lupus treatment.[17] Cutaneous Lupus Erythematosus Disease Area and Severity Index evaluates the cutaneous disease activity (CLASI-A) (erythema, scale/hypertrophy, mucocutaneous involvement, recent hair loss, and nonscarring alopecia) and also the disease-induced damage (CLASI-D) (depigmentation, scarring, atrophy/panniculitis, and scarring alopecia).[17] The severity of the involvement of each signal in distinct areas is documented according to the most severely affected region. The CLASI-A and CLASI-D scores range from 0 to 70 and 0 to 56, respectively, with higher scores indicating more severe disease.[17] Jolly et al.[23] showed a correlation of CLASI-A and CLASI-D with cutaneous lupus activity and damage assessed by the physician by visual analog scale in patients with SLE/CLE.

To our knowledge, only 1 study evaluated the efficacy of thalidomide for the treatment of cutaneous SLE/CLE activity through the CLASI. Cortés-Hernández et al.[1] evaluated the medical records of 60 patients with SLE/CLE in Spain, who were treated with thalidomide (initial dose of 100 mg/d and cumulative mean dose of 16.5 ± 12.6 g). Complete response (defined as CLASI-A = 0) occurred in 85% of patients, already noticeable after 2 weeks of medication use and with an average of 8 weeks until the achievement of a complete response. Furthermore, partial response (decrease of more than 50% in the CLASI-A value after at least 3 months of treatment) was observed in 14% of cases. However, relapses (increase in CLASI-A ≥2 points) occurred in 70% of patients after 2 to 60 months of drug withdrawal. Complete response was reachieved after the reintroduction of thalidomide.[1]

Specialists' Approach to Cutaneous Assessment

The CLASI, the only validated tool to assess specifically cutaneous activity and damage in SLE/CLE,[17] should be preferred over the Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index scores because these 2 instruments only report the presence or absence of these conditions, but do not quantify the relevant parameters for drug response.[24,25] Of note, Krathen et al.[26] showed that rheumatologists, physicians who commonly follow SLE patients, have enough skills to perform CLASI adequately.