Key Points
➤ Interstitial lung disease (ILD) is a significant cause of morbidity and
mortality in patients with systemic sclerosis (SSc).
➤ The standard of care for patients with SSc includes lifestyle
modification measures to minimize risks for overt and/or occult micro-
aspiration, with or without treatment with proton pump inhibitors
(PPI), H2-blockers, and/or prokinetic agents.
➤ Associated manifestations of SSc not addressed in this guideline
include pulmonary hypertension, esophageal dysmotility, and
gastroesophageal reflux disease (GERD).
➤ ILD is defined as the radiologic presence of reticulation, traction
bronchiectasis, traction bronchiolectasis, honeycomb cysts,
ground glass opacities/air space consolidation, other interstitial
lung abnormalities, or any of the recognized patterns of interstitial
pneumonias (usual interstitial pneumonia (UIP), probable UIP,
indeterminate for UIP; non-specific interstitial pneumonia (NSIP),
organizing pneumonia (OP), lymphoid interstitial pneumonia (LIP),
pleuroparenchymal fibroelastosis (PPFE), or unclassifiable interstitial
pneumonias) reported in the context of SSc.
