The systemic management of cutaneous dermatomyositis: Results of a stepwise strategy.

Treatment of dermatomyositis (DM) is often achieved with a stepwise algorithm. However, the literature lacks quality evidence to support the use of this therapeutic strategy. The result of a stepwise therapeutic strategy in the management of skin-only DM is presented to better understand the clinical outcomes and allow for future studies. A cohort of 102 patients with DM, 41 of whom had skin-only disease, were seen between July 2009 and April 2013 at a referral-based connective tissue disease clinic. The Cutaneous Dermatomyositis Disease Area and Severity Index was used to prospectively assess disease severity and the outcomes in 41 adult patients with skin-only DM were analyzed. Of the 41 patients with skin-only DM, 23 patients (56.1%) received antimalarial medications alone and 18 patients (43.9%) received second- or third-line agents. Ten patients (24.4%) remained at the first level of the treatment algorithm and received only hydroxychloroquine. Prednisone was included in the treatment regimen for 11 patients with skin-only disease (26.8%). The results show that management of cutaneous DM often requires second-line agents because antimalarial medications alone are insufficient to treat most patients with skin-only disease.

Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index: characterizing disease severity and assessing responsiveness to clinical change.

BACKGROUND: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) was developed for use in clinical trials and longitudinal patient assessment. OBJECTIVES: To characterize disease severity using the CDASI and assess the responsiveness of this instrument to clinically meaningful changes in disease activity. METHODS: Patients with cutaneous dermatomyositis at the University of Pennsylvania (UPenn, n = 93) and Stanford University (Stanford, n = 106) were prospectively evaluated using the CDASI, physician global assessment (PGA) Likert scales and a visual analogue scale (VAS). Data was analysed using logistic regression models and receiver operating characteristic curves to select cut-offs. RESULTS: Baseline CDASI activity scores for the patients evaluated at UPenn ranged from 0 to 47 (median 17), and baseline PGA VAS scores ranged from 0 to 9·6 (median 1·1). At UPenn a CDASI activity score of 19 differentiated mild from moderate and severe disease. At Stanford baseline CDASI scores ranged from 0 to 48 (median 21), baseline PGA VAS scores ranged from 0 to 9·7 (median 4·2) and CDASI activity scores of 14 or less characterized mild disease. When a 2-cm change in the PGA VAS was regarded as a clinically significant improvement, a 4-point (UPenn) or 5-point (Stanford) change in CDASI reflected a minimal clinically significant response. CONCLUSIONS: The CDASI is a valid and responsive measure that can be used to characterize cutaneous dermatomyositis severity and detect improvement in disease activity. Variations in cut-offs may be due to differences in disease severity between the two populations or inter-rater variations in the use of the external gold measures.