The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity score is a validated skin-specific outcome measure used to determine the longitudinal course of disease in patients with the condition, according to the findings of a recent retrospective cohort study published in the Journal of the American Academy of Dermatology.
Patients in whom the CDASI activity subscore was recorded for ≥2 years were included in the study. Disease progression was classified as improved, worsened, or stable; disease course was characterized as monophasic, polyphasic, or chronic. Variability in disease activity over time was assessed by manually computing the number of flares divided by the number of years of follow-up (ie, flares per year).
A total of 40 patients with cutaneous dermatomyositis met the study criteria. Participants were divided into mild (n=16) or moderate to severe (n=24) baseline severity groups. Mean patient age was 53.8 years; most of the patients were women (90%) and white (95%). Disease subtype was characterized as classic in 52.5% of patients and as skin predominant in the remaining 47.5%. Median follow-up from initial visit was 3.42 years.
The majority of patients demonstrated improvement in disease activity (52.5%; 21 of 40) compared with those with worsening of disease activity (17.5%; 7 of 40) or those with stable disease (30.0%; 12 of 40). Although most of the participants with mild disease remained stable (62.5%; 10 of 16), most of those with moderate to severe disease improved (79.2%; 19 of 24).
A polyphasic disease course was observed in the majority of patients (82.5%; 33 of 40) compared with a monophasic course (12.5%; 5 of 40) or a chronic course (5%; 2 of 40). The average number of disease flares reported per year was independent of baseline disease activity.
The investigators concluded that baseline CDASI activity scores are associated with particular patterns of disease course and disease progression in patients with cutaneous dermatomyositis. The need exists for additional research in this area in order to evaluate how treatment modalities affect disease outcomes over time in patients with this debilitating condition.
Chansky PB, Olazagasti JM, Feng R, Werth VP. Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) [published online October 21, 2017]. J Am Acad Dermatol. doi:10.1016/j.jaad.2017.10.022