Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis

Abstract

To investigate the longitudinal association of nailfold capillary density (NCD; as a potential marker of activity) with various other clinical measures of disease activity and to evaluate baseline NCD as a predictor of disease outcome in children with JDM. Data from 809 clinic visits from 92 JDM patients were prospectively collected at each clinic visit over a time period of 5.5 years. The number of capillaries per millimetre at the distal nailfold was scored using a stereomicroscope. Disease activity was determined using the Childhood Myositis Assessment Scale (CMAS) and a modification of the validated disease activity score (DAS), which included three skin (SDAS) and three muscle (MDAS) criteria. An inception cohort subgroup (n=28) with a baseline visit at diagnosis was analysed separately. Both DAS subscores, MDAS (β = -0.04437, P < 0.0001) and SDAS (β = -0.1589, P < 0.0001), as well as the CMAS (β = 0.02165, P < 0.0001) were significantly associated with loss of end row nailfold capillary over time (multiple regression mixed-model analysis). All patients in the inception subcohort showed a reduced baseline NCD (diagnostic sensitivity = 100%) that improved as the disease improved, but this did not predict longer term outcome or course of disease. NCD is a marker of skin and muscle disease activity, and is an important measure of disease activity changes from visit to visit. Determination of capillary density may be useful when making treatment decisions. A decrease in NCD may be considered for inclusion in the diagnostic criteria due to its high sensitivity.