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. 2010 Oct;36(4):893-900.
doi: 10.1183/09031936.00139809. Epub 2010 Mar 29.

Systemic sclerosis and bilateral lung transplantation: a single centre experience

R Saggar  1 , D Khanna, D E Furst, J A Belperio, G S Park, S S Weigt, B Kubak, A Ardehali, A Derhovanessian, P J Clements, S Shapiro, C Hunter, A Gregson, M C Fishbein, J P Lynch Iii, D J Ross, R Saggar
Affiliations

Systemic sclerosis and bilateral lung transplantation: a single centre experience

R Saggar et al. Eur Respir J. 2010 Oct.

Abstract

Lung involvement is the leading cause of death in systemic sclerosis (SSc), but lung transplantation (LT) for systemic disease remains controversial. Our objective was to comprehensively evaluate post-LT outcomes for SSc compared to idiopathic pulmonary fibrosis (IPF). We retrospectively evaluated bilateral LT recipients (LTRs) with SSc or IPF at our centre between January 1, 2003 and December 31, 2007. The primary end-point was all-cause mortality at 1 yr post-LT. Secondary end-points included assessments of acute rejection (AR), pulmonary function, infection and chronic rejection. 14 patients with SSc and 38 patients with IPF underwent LT. Apart from a younger SSc cohort (53.2 versus 58.8 yrs; p = 0.02), the two groups were well matched. 1-yr all-cause mortality was no different between SSc (6.6%) and IPF (13.1%) groups, after adjusting for age (p = 0.62). Rates of (AR) ≥2 were significantly increased for the SSc compared with the IPF group (hazard ratio (HR) 2.91; p = 0.007). Other end-points, including chronic rejection, infection and pulmonary function, showed no differences. SSc LTRs experience similar survival 1 yr post-LT when compared to IPF. AR rates may be significantly higher in the SSc group. Longer follow-up is necessary to determine the effects of gastrointestinal dysfunction and AR on late allograft function in SSc LTR.

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Figures

Figure 1
Figure 1
Kaplan-Meier plot displaying the estimated time to first acute rejection ≥1 for the idiopathic pulmonary fibrosis group [indicated by a solid line (—)] vs. the systemic sclerosis group [indicated by a dashed line (---)], during the first year after lung transplantation. There is no significant difference between groups (log-rank test: p=0.06).
Figure 2
Figure 2
Kaplan-Meier plot displaying the estimated time to first acute rejection ≥2 for the idiopathic pulmonary fibrosis group [indicated by a solid line (—)] vs. the systemic sclerosis group [indicated by a dashed line (---)], during the first year after lung transplantation. There is a significant difference between groups (log-rank test: p=0.007).
Figure 3
Figure 3
Kaplan-Meier plot displaying freedom from chronic rejection (BOS) post lung transplantation for the idiopathic pulmonary fibrosis group [indicated by a solid line (—)] vs. the systemic sclerosis group [indicated by a dashed line (---)]. For the first five years after lung transplantation, there is no significant difference between the two groups (Log-rank; p=0.14).
Figure 4
Figure 4
Kaplan-Meier plot displaying the survival estimates for the idiopathic pulmonary fibrosis group [indicated by a solid line (—)] vs. the systemic sclerosis group [indicated by a dashed line (---)]. There was no significant difference in overall survival between groups (log-rank test: p=0.69).
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