How Is Pulmonary Function Assessed in Patients With Juvenile Systemic Sclerosis? Do We Have a Good Clinical Standard?

Juvenile systemic sclerosis (jSSc) is a rare but potentially life-threatening disease, with a prevalence of 3 to 4 in 1,000,000 children. Similar to that in adults, 1 interstitial lung disease (ILD) is the major cause of morbidity in children with systemic sclerosis (SSc) and occurs at similar prevalences. 2 .