Systemic Sclerosis-Associated Interstitial Lung Disease

Publication Date: September 29, 2023

Key Points

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.

Treatment

...reatment

...recommendation for the use of the...


...clophosphamide (Low, Conditional R...

...uximab (Very low, Conditional Recommend...

...(Very low, Conditional Recommendation)3094...

...ib (Very low, Conditional Recommenda...

...mycophenolate (Very low, Conditional...


...ne (Very low, Strong Recommendation)3...

...ne plus mycophenolate (Very low, St...


Caution must be taken in using systemic corti...


...mmary of Treatment Recommendations for...