Diffuse cutaneous juvenile systemic sclerosis, with rapid progression and favorable initial response to tocilizumab: report of a clinical case

Abstract

Juvenile systemic sclerosis is a multisystem connective tissue disease characterized by skin fibrosis, vasculopathy, and internal organ involvement. In pediatric patients, it has a variety of clinical manifestations, many of which are different from adult forms. The diffuse cutaneous form predominates; however, the involvement of organs, at least symptomatically, is less than in adults. Its diagnosis is based on clinical criteria and specific antibodies. Treatment must be early and individualized according to the complications of each patient. In recent years, in refractory cases, the use of Tocilizumab (TCZ) has shown promising results with improvements in skin and lung involvement. A clinical case is presented where this scenario is exposed.