2006
DOI: 10.1002/art.22264
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Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension

Abstract: Objective. Lung transplantation is a viable, lifesaving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention.Methods. Survival following lung transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pul… Show more

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Cited by 158 publications
(73 citation statements)
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“…Importantly, pulmonary complications, including pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), are the leading causes of SSc-related death (3). PAH is a progressive disease leading to increased pulmonary vascular resistance and eventual right heart failure and death (4).…”
Section: Methodsmentioning
confidence: 99%
“…Importantly, pulmonary complications, including pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), are the leading causes of SSc-related death (3). PAH is a progressive disease leading to increased pulmonary vascular resistance and eventual right heart failure and death (4).…”
Section: Methodsmentioning
confidence: 99%
“…These patients should be considered for lung transplantation early in the course of their disease, in the absence of other significant comorbidity (40). Patients with SSc have long been considered suboptimal candidates for lung or heart-lung transplantation.…”
Section: Le Pavec Et Almentioning
confidence: 99%
“…Presumed heightened risk in the postoperative period, arising from SSc-related gastroesophageal reflux, renal impairment, or skin fibrosis, likely contribute to this perception. However, the results of 3 recent studies (40)(41)(42) suggest that in carefully selected patients, lung transplantation may represent a viable therapeutic option in those with end-stage lung dysfunction resulting from SSc.…”
Section: Le Pavec Et Almentioning
confidence: 99%
“…Once thought to be prohibitive because of an unacceptable mortality rate, lung transplant in SSc ILD has now been shown to have a similar prognosis for lung transplant for IPF [63][64][65][66]. However, these comparable survival rates require that the recipients are carefully selected.…”
Section: Lung Transplantmentioning
confidence: 99%