title: Systemic sclerosis and bilateral lung transplantation: a single centre experience.
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.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Dept of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095-1690, USA. rsaggar@mednet.ucla.edu
authors.forename: R
authors.initials: R
authors.lastname: Saggar
authors.forename: D
authors.initials: D
authors.lastname: Khanna
authors.forename: D E
authors.initials: DE
authors.lastname: Furst
authors.forename: J A
authors.initials: JA
authors.lastname: Belperio
authors.forename: G S
authors.initials: GS
authors.lastname: Park
authors.forename: S S
authors.initials: SS
authors.lastname: Weigt
authors.forename: B
authors.initials: B
authors.lastname: Kubak
authors.forename: A
authors.initials: A
authors.lastname: Ardehali
authors.forename: A
authors.initials: A
authors.lastname: Derhovanessian
authors.forename: P J
authors.initials: PJ
authors.lastname: Clements
authors.forename: S
authors.initials: S
authors.lastname: Shapiro
authors.forename: C
authors.initials: C
authors.lastname: Hunter
authors.forename: A
authors.initials: A
authors.lastname: Gregson
authors.forename: M C
authors.initials: MC
authors.lastname: Fishbein
authors.forename: J P
authors.initials: JP
authors.lastname: Lynch Iii
authors.forename: D J
authors.initials: DJ
authors.lastname: Ross
authors.forename: R
authors.initials: R
authors.lastname: Saggar
mesh_terms: D000328:Adult; D000465:Algorithms; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology; D016040:Lung Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D010349:Patient Compliance; D011658:Pulmonary Fibrosis / Q000150:complications / Q000628:therapy; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents