Major lung complications of systemic sclerosis

Nat Rev Rheumatol. 2018 Sep;14(9):511-527. doi: 10.1038/s41584-018-0062-0.


Systemic sclerosis (SSc) is associated with high mortality owing to internal organ complications, and lung disease is the leading cause of SSc-associated death. The most notable lung complications in SSc are fibrosis and pulmonary arterial hypertension (PAH). A major challenge for the management of lung disease in SSc is detecting those patients with severe pathology and those patients who are likely to benefit from available treatments. In the past few years, strategies for managing lung fibrosis and pulmonary hypertension, including PAH, have greatly progressed. For lung fibrosis, the tools to assess risk of progression and severity of the disease have been refined. Clinical trial results support the use of immunosuppression, including high-intensity regimens with autologous stem cell transplantation. New trials are underway to test other potential therapies including treatments that are approved for use in idiopathic lung fibrosis. For PAH, identifying individuals at high risk of disease development is critical. In addition, individuals who have borderline elevation of pulmonary arterial pressure need to be appropriately managed and followed up. Many approved drugs targeting PAH are now available, and results from large-scale clinical trials provide robust evidence that various treatments for SSc-associated PAH are associated with good long-term outcomes.

Publication types

  • Review

MeSH terms

  • Clinical Trials as Topic
  • Disease Progression
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / therapy
  • Immunosuppressive Agents / therapeutic use
  • Pulmonary Fibrosis / diagnosis*
  • Pulmonary Fibrosis / etiology
  • Pulmonary Fibrosis / mortality
  • Pulmonary Fibrosis / therapy
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / mortality
  • Scleroderma, Systemic / therapy
  • Severity of Illness Index
  • Stem Cell Transplantation
  • Transplantation, Autologous
  • Treatment Outcome


  • Immunosuppressive Agents