title: Lung transplantation and systemic sclerosis.
abstract: We performed lung transplantation in nine patients with Scleroderma related lung disease. Patient characteristics included: 7 (78%) females, 6 (67%) with limited and 3 (33%) with diffuse Scleroderma. Pulmonary fibrosis was present in 7 (78%) and pulmonary hypertension in 4 (44%). All patients were carefully screened by the Johns Hopkins and University of Maryland Scleroderma Center and only referred for transplantation when concomitant renal insufficiency (creatinine clearance < or = 50 ml/min), aspiration, and skin brakdown were excluded. When compared to a similar group of transplant patients with nonscleroderma lung disease (primary pulmonary fibrosis), there was no significant difference in post-transplant survival at four years (76.2 +/- 0.15% vs. 69.2% +/- 0.12%), mean annual incidence rate for acute rejection (0.14 +/- 0.14 vs. 0.47 +/- 0.13) and infection (viral 0.17 +/- 0.17 vs. 0.29 +/- 0.11) (bacterial 0.17 +/- 0.17 vs. 1.4 +/- 0.4) (fungal 0.99 +/- 0.69 vs. 0.36 +/- 0.16) or serum creatinine (1.55 +/- 0.34 mg/dl vs. 1.15 +/- 0.09 mg/dl). We conclude that lung transplantation is viable option for carefully selected patients with scleroderma related lung disease.
authors.affiliation: John Hopkins Hospital, Division of Pulmonary and Critical Care Medicine, Baltimore, Maryland, USA.
authors.forename: V
authors.initials: V
authors.lastname: Rosas
authors.forename: J V
authors.initials: JV
authors.lastname: Conte
authors.forename: S C
authors.initials: SC
authors.lastname: Yang
authors.forename: S P
authors.initials: SP
authors.lastname: Gaine
authors.forename: M
authors.initials: M
authors.lastname: Borja
authors.forename: F M
authors.initials: FM
authors.lastname: Wigley
authors.forename: B
authors.initials: B
authors.lastname: White
authors.forename: J B
authors.initials: JB
authors.lastname: Orens
mesh_terms: D000075202:Contraindications; D005260:Female; D005767:Gastrointestinal Diseases / Q000601:surgery; D006331:Heart Diseases / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery; D007674:Kidney Diseases / Q000601:surgery; D008171:Lung Diseases / Q000601:surgery*; D016040:Lung Transplantation / Q000009:adverse effects; D008297:Male; D011658:Pulmonary Fibrosis / Q000601:surgery; D012595:Scleroderma, Systemic / Q000503:physiopathology / Q000601:surgery*