title: [Lung involvement in systemic sclerosis].
abstract: Systemic sclerosis (SSc) is a protean disorder in which prognosis and treatment are tailored on the basis of organ involvement. Among SSc lung manifestations, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) or the combination of both, are the first cause of SSc mortality and impact heavily on patient quality of life. ILD may begin early in disease and usually progresses slowly. However, approximately 10% of patients with ILD may reach terminal respiratory insufficiency. PAH may be an early or late complication of SSc in which increased blood pressure in pulmonary arteries leads to right heart failure. Current treatments provide some benefit, but both SSc-ILD and PAH still represent an enormous unmet need of more efficacious therapeutic strategies.
authors.forename: M
authors.initials: M
authors.lastname: Posa
authors.forename: M
authors.initials: M
authors.lastname: Stelle
authors.forename: F
authors.initials: F
authors.lastname: Lador
authors.forename: C
authors.initials: C
authors.lastname: Chizzolini
mesh_terms: D001794:Blood Pressure; D018450:Disease Progression; D006301:Health Services Needs and Demand; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D017563:Lung Diseases, Interstitial / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D011379:Prognosis; D011788:Quality of Life; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality / Q000628:therapy