Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation.

BACKGROUND: The use of lung transplantation (LTX) to treat respiratory failure because of scleroderma is controversial. We present our experience, review the current literature, and suggest specific criteria for LTX in scleroderma. Of the 174 patients who underwent LTX at our center, seven (4%) had scleroderma-associated respiratory failure.

PATIENTS AND METHODS: A MEDLINE search of the English literature was performed for studies of LTX in patients with scleroderma between 1986 and 2006. A Kaplan-Meier survival curve was calculated over the time of the studies.

RESULTS: The MEDLINE search yielded one large review and four small case series. The small case series were included in the review. The review and our series yield a total of 54 patients. Mean patient age was 47.1 yr; 59.3% were female. Pre-operative lung data were available for 24 patients: 22 (92%) had pulmonary fibrosis and 17 (71%) had pulmonary hypertension. Most patients (69%) underwent single-lung transplantation. Mean forced expiratory volume at one s after LTX was 67% (range 56-87%). There was no difference in infection and rejection rates between the patients with scleroderma and other LTX recipients. The two- and five-yr survival rates were 72% and 55%, respectively.

CONCLUSIONS: LTX is a valid option in well-selected patients with scleroderma and pulmonary fibrosis, yielding good pulmonary function and acceptable morbidity and mortality.