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Scleroderma Lung Disease

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Abstract

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.

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Authors and Affiliations

  1. Université Paris-Sud 11, Centre National de Référence de l’Hypertension Artérielle Pulmonaire, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Assistance Publique, Hôpitaux de Paris, 157 rue de la Porte de Trivaux, 92140, Clamart, France

    Jérôme Le Pavec & Marc Humbert

  2. Division of Pulmonary and Critical Care Medicine, 1830 E. Monument St, Fifth Floor, Baltimore, MD, 21205, USA

    Jérôme Le Pavec, Stephen C. Mathai & Paul M. Hassoun

  3. Université Lille 2. Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Lille, France

    David Launay

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  1. Jérôme Le Pavec
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  2. David Launay
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Correspondence to Jérôme Le Pavec.

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Le Pavec, J., Launay, D., Mathai, S.C. et al. Scleroderma Lung Disease. Clinic Rev Allerg Immunol 40, 104–116 (2011). https://doi.org/10.1007/s12016-009-8194-2

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