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 screned by the Johns Hopkins and University of Maryland Scleroderma Center and only referred for transplantation '{'(hen concomitant renal insufficiency (creatinine clearance :$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:t0.15% vs. 69.2%:t0.12%), mean annual incidence rate for acute rejection (0.14:t0.14 vs. OA7:t0.13) and infection (viral 0.17:t0.17 vs. 0.29:t0.11) (bacterial 0.17:t0.17 vs. 1.4:tOA) (fungal 0.99:t0.69 vs. 0.36:t0.16) or serum creatinine (1.55 :to.34 mg/dl vs. 1.15 :to.09 mg/dl). We conclude that lung transplantation is viable option for carefuly selected patients with scleroderma related lung disease.

Keywords: Lung Transplantation, systemic sclerosis, scleroderma