Scleroderma lung disease

Jérôme Le Pavec, David Launay, Stephen C. Mathai, Paul M. Hassoun, Marc Humbert

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Pulmonary involvement is second in frequency only to esophageal involvement as a visceral complication of systemic sclerosis (SSc) and has surpassed renal involvement as the most common cause of death. Interstitial lung disease and pulmonary vascular disease, particularly pulmonary arterial hypertension, are the most commonly encountered types of lung involvement. Chronic aspiration, airway disease, neuromuscular weakness, extrinsic pulmonary restrictive pathology, pleural effusions, pneumothorax, and lung cancer cause clinically significant disease and occur commonly enough to be routinely considered in the assessment of the SSc patient with respiratory symptoms. Affected patients have a significantly worse prognosis than patients with SSc who are free of pulmonary involvement.

Original languageEnglish (US)
Pages (from-to)104-116
Number of pages13
JournalClinical Reviews in Allergy and Immunology
Issue number2
StatePublished - Apr 2011


  • Interstitial lung disease
  • Pulmonary arterial hypertension
  • Scleroderma
  • Scleroderma lung disease
  • Scleroderma-related pulmonary arterial hypertension

ASJC Scopus subject areas

  • Immunology and Allergy


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