Clinical and ultrasonographic evaluation of disease activity in juvenile localized scleroderma: a prospective study

Abstract

The early diagnosis of rheumatic diseases in childhood with the establishment of criteria diagnostic and evaluating the clinical activity/remission disease has improved the prognosis, morbidity and mortality of these diseases. These diseases include juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE), juvenile dermatomyositis (JDM), autoimmune vasculitis, autoinflammatory syndromes and scleroderma. However, in some diseases such as scleroderma, there are difficulties in determining activity parameters and there is no validation of these for the pediatric population.Juvenile localized scleroderma is a disease characterized by fibrosis of the skin and subcutaneous tissue. Ultrasound may be useful in the diagnosis and monitoring of the skin involvement and allows the assessment of the depth and the extension of sclerosis in the affected region. Hyperemia and hyperechogenicity reflect activity of the lesions. Due to the scarcity of studies with ultrasound in skin lesions of patients with juvenile localized scleroderma and the importance of this exam in evaluating disease activity even before the clinical lesions occur, we decided to perform this study. We propose to evaluate the ultrasound findings in patients with juvenile localized scleroderma, the correlation of clinical and ultrasonographic activity and the response to treatment in these patients. In this multicenter study (Universidade Federal de São Paulo - UNIFESP e Instituto da Criança da Universidade de São Paulo - ICR/FMUSP), 60 localized juvenile scleroderma patients and 30 matched controls will be prospectively evaluated through a two-time clinical and ultrasound evaluation a year apart. The pediatric rheumatologist will blindly assess skin changes and disease activity through specific scores. Ultrasound evaluation will be performed by a blinded professional, taking into account the thickness and echogenicity of the dermis in affected regions and healthy areas. (AU)