Response to hydroxychloroquine in Japanese patients with lupus-related skin disease using the cutaneous lupus erythematosus disease area and severity index (CLASI).

BACKGROUND: Hydroxychloroquine (HCQ) is not available in Japan. To design a clinical trial of HCQ, we evaluated the response to HCQ in Japanese patients with lupus-related skin disease using the cutaneous lupus erythematosus disease area and severity index (CLASI). METHODS: Twenty-seven patients with lupus-related skin disease who started HCQ at four hospitals were included. Patients were categorized into responders by the CLASI response criteria. The points and the rate of improvement in the CLASI activity score after 16 weeks of treatment were analyzed, focusing on six parameters: systemic lupus erythematosus (SLE), skin manifestations, disease duration, prednisolone, smoking, and severity. RESULTS: Twenty-seven patients, including 17 with SLE (6 with SLE/Sjögren's syndrome), were analyzed retrospectively. Twenty-three patients (85 %) were categorized as responders. The mean CLASI activity score improved from 10.1 to 4.5 (p < 0.0001). The improvement rate did not differ in these parameters except for that of annular erythema (81.6 versus 34.3 %, p = 0.036). On multivariate analysis, the baseline CLASI activity score (CLASI ≥9) correlated with the greatest decrease in CLASI activity score (F = 69.7, p < 0.0001). CONCLUSIONS: CLASI is a reliable indicator to evaluate the efficacy of the drug, and HCQ is an effective treatment for Japanese patients with lupus-related skin disease.

Experience with the use of hydroxychloroquine for the treatment of lupus erythematosus.

Hydroxychloroquine (HCQ) is generally used to treat systemic lupus erythematosus (SLE) in Western countries. However, chloroquine retinopathy became a problem in Japan, and chloroquine has never been used since then. Even now HCQ remains non-approved. Therefore, the Japanese Hydroxychloroquine Study Group has been organized, and activities have started to have HCQ approved within Japan. In the present study, we investigated the effectiveness of HCQ against the skin manifestations of lupus erythematosus. There were seven patients, all female, and they consisted of four patients with SLE (skin lesion type: discoid lupus erythematosus [DLE] in three, subacute cutaneous lupus erythematosus in one and lupus erythematosus profundus in one), two patients with cutaneous lupus erythematosus (both DLE), and one patient with a combination of SLE and dermatomyositis. HCQ was effective in three patients and ineffective in the two patients. We could not judge the efficacy of HCQ in the other two patients. There were no adverse effects in any of the patients. Efficacy was exhibited against telangiectasia and erythema. HCQ is also an effective and safe treatment for Japanese patients, and it is hoped that it will be approved for use in Japan very soon.