Scleroderma Lung Disease |
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Authors: | Jérôme Le Pavec David Launay Stephen C Mathai Paul M Hassoun Marc Humbert |
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Institution: | 1.Université Paris-Sud 11, Centre National de Référence de l’Hypertension Artérielle Pulmonaire, Service de Pneumologie et Réanimation Respiratoire, H?pital Antoine-Béclère, Assistance Publique, H?pitaux de Paris,Clamart,France;2.Division of Pulmonary and Critical Care Medicine,Baltimore,USA;3.Université Lille 2. Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, H?pital Claude-Huriez,Lille,France |
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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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Keywords: | |
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