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Contains fulltext : 282871.pdf (Publisher’s version ) (Open Access) BACKGROUND: The diagnosis of Scleroderma En Coup de Sabre (ECDS)/Parry Romberg Syndrome (PRS) is mainly based on characteristic clinical findings. Methods to objectively monitor the course of the disease in a standardized way are lacking. OBJECTIVES: This descriptive, retrospective, single centre cohort study aims to describe the contribution of 3D photographs in the assessment of the degree of facial asymmetry changes over time in growing children and adolescents with ECDS and PRS. METHODS: Six patients diagnosed with ECDS/PRS, with a follow-up period of at least 24 months and at least three 3D photographs were included. Mirroring these 3D photographs was automatically performed using surface-based matching to generate a colour-coded distance map, illustrating the inter-surface distance and thereby asymmetry between the original and mirrored 3D photographs. The percentage of absolute distances between the original and mirrored 3D photograph were calculated. RESULTS: In two patients, impressive decreases in the percentages of absolute distance levels over time were found, whereas the other patients did not show progression of asymmetry over time. CONCLUSION: This study shows the potential of 3D stereophotogrammetry as an objective tool to measure disease activity over time in patients with ECDS/PRS.

We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM).Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT) and quantitative muscle echo intensity (EI) were assessed with QMUS in 4 muscles.Six patients experienced a monocyclic course. At diagnosis EI was slightly increased, and MT was relatively normal. After start of treatment MT first decreased and EI increased, with normalization of EI within 6-12 months (n = 4). One patient had higher EIs at diagnosis and slower normalization, indicating fibrosis, despite early normalization of CMAS. One patient experienced a chronic course, with high EIs and atrophy during follow-up.QMUS can provide additional information for follow-up of JDM regarding disease severity and residual muscle damage, particularly after normalization of CMAS.