Multimodality thoracic imaging of juvenile systemic sclerosis: emphasis on clinical correlation and high-resolution CT of pulmonary fibrosis

Abstract

OBJECTIVE. Juvenile systemic sclerosis is a rare multisystem autoimmune disorder characterized by vasculopathy and multiorgan fibrosis. Cardiopulmonary complications are the leading cause of morbidity and mortality. Although pulmonary fibrosis is the complication that is most common and well described, cardiovascular and esophageal involvement may also be observed. In this article, common thoracic findings in juvenile systemic sclerosis will be discussed. We will focus on chest CT, including CT findings of pulmonary fibrosis and associated grading methods, as well as cardiac MRI and esophageal imaging. CONCLUSION. Radiologists play a pivotal role in the initial diagnosis and follow-up evaluation of pediatric patients with systemic sclerosis. Treatment decisions and prognostic assessment are directly related to imaging findings along with clinical evaluation.

Keywords: cardiac MRI; fibrosis grading systems; gastroesophageal reflux; high-resolution CT; juvenile systemic sclerosis; pulmonary arterial hypertension; pulmonary fibrosis.