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Abstract Number: 1797

One-Year Survival of Adults with Systemic Sclerosis Following Lung Transplantation: A Nationwide Cohort Study

Elana J. Bernstein1, Eric R. Peterson2, Joan M. Bathon2 and David J. Lederer2, 1Rheumatology, Columbia University College of Physicians & Surgeons, New York, NY, 2Medicine, Columbia University College of Physicians & Surgeons, New York, NY

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: interstitial lung disease, pulmonary fibrosis, Scleroderma, systemic sclerosis and transplantation

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Session Information

Session Title: ACR Plenary Session II: Discovery 2014

Session Type: Plenary Sessions

Background/Purpose: Lung transplantation is a potentially life-saving treatment for patients with systemic sclerosis (SSc) who have developed end-stage lung disease due to interstitial lung disease and/or pulmonary hypertension. However, many transplant programs are hesitant to offer lung transplantation (LTx) to those with SSc due to concerns about extra-pulmonary involvement that might affect short- and long-term survival. However, survival data for lung transplantation in SSc are sparse. The primary aim of this study was to determine whether adults with SSc have higher 1-year mortality rates after LTx compared to those with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) not due to SSc. We hypothesized that adults with SSc would have higher 1-year mortality rates after LTx than those with ILD or PAH not due to SSc.

Methods: We performed a retrospective cohort study of adults who underwent double or single LTx in the United States between May 4, 2005 (the date of implementation of the lung allocation score) and September 14, 2012. Data were provided by the United Network for Organ Sharing, a non-profit organization that records data on all solid organ transplants performed in the US. Subjects were included if they were at least 18 years of age at the time of LTx; had a diagnosis of SSc, ILD, or PAH; and were transplanted at a center that has performed at least 1 LTx for SSc. Subjects were excluded if they had received a heart-lung transplant; if they received a LTx from a living donor; or if they had missing data on survival time. We modeled diagnosis (SSc) as the independent binary variable of interest in stratified Cox regression models where survival time was the dependent variable, adjusting for recipient, donor, and procedural factors (Table 1). We used multiple imputation to account for missing covariate data.

Results: A total of 3763 adults were transplanted during the study period and met inclusion criteria: 229 with SSc, 201 with PAH, and 3333 with ILD (Table 1). The 1-year unadjusted mortality rate following LTx per 100 person-years was 21.4 among adults with SSc, 19.0 among adults with PAH, and 17.8 among adults with ILD. A diagnosis of SSc was associated with a multivariable-adjusted 48% relative increase in the 1-year mortality rate compared to a diagnosis of ILD (HR 1.48, 95% CI 1.01-2.17). However, a diagnosis of SSc was not associated with a relative increase in the 1-year mortality rate compared to a diagnosis of PAH (HR 0.85, 95% CI 0.50-1.44).

Conclusion: Adults with SSc had a 48% increased risk of death at 1 year following LTx compared to adults with ILD, but no increase in risk of death at 1 year compared to adults with PAH. Rather than denying SSc patients LTx because of their SSc diagnosis, variables need to be identified that will enable risk stratification of these patients prior to LTx, with particular attention to modifiable risk factors.

 

Table 1: Recipient Characteristics and Covariates

Recipient Characteristics

SSc

N = 229

PH

N = 201

ILD

N = 3333

Age, years

53 (44-59)

46 (34-57)

62 (56-66)

Female sex

135 (58.95%)

126 (62.69%)

941 (28.23%)

Race/Ethnicity

 

 

 

     White

162 (70.74%)

161 (80.10%)

2782 (83.47%)

     Black

38 (16.59%)

17 (8.46%)

199 (5.97%)

     Hispanic

25 (10.92%)

17 (8.46%)

261 (7.83%)

     Asian

3 (1.313%)

5 (2.49%)

67 (2.01%)

     Other

1 (0.44%)

1 (0.50%)

24 (0.72%)

LAS score

44.31 (38.03-52.48)

36.90 (33.93-46.00)

45.36 (39.42-58.10)

Height, cm

168.97 (10.29)

169.59 (9.62)

172.28 (9.56)

Body mass index (kg/m2)

25.10 (4.21)

24.93 (4.36)

27.16 (3.99)

Steroid use

117 (52.00%)

N = 225

45 (23.20%)

N = 194

1741 (53.75%)

N = 3239

Pulmonary artery systolic pressure, mmHg

48 (37-66)

N = 223

83 (68-98)

N = 194

39 (32-48)

N = 3206

Forced vital capacity, %predicted

44 (34-60)

N = 225

73 (60-87)

N = 197

45 (36-57.5)

N = 3300

Creatinine, mg/dL

0.8 (0.7-1.0)

1.0 (0.8-1.2)

0.9 (0.7-1.0)

N = 3322

Extracorporeal membrane oxygenation

11 (4.80%)

5 (2.49%)

50 (1.50%)

Mechanical ventilation

23 (10.04%)

6 (2.99%)

232 (6.96%)

Oxygen requirement, L/min

5 (3-6)

N = 228

 

4 (2-6)

N = 200

4 (3-6)

N = 3310

Covariates adjusted for in Cox regression models

Recipient factors

Age; Sex; Race/Ethnicity; LAS score; Height; BMI; Steroid use; Pulmonary artery systolic pressure; Forced vital capacity; Creatinine; Extracorporeal membrane oxygenation; Mechanical ventilation

Donor factors

Age; Sex; Height; Body mass index; PaO2 on FiO2 of 1.0; Pulmonary infection; ≥ 20 Pack-years smoking; Heavy alcohol use; Cause of death

Procedural factors

Ischemic time; Single vs. bilateral transplant; Transplant center; Distance from donor hospital to transplant center; Recipient-donor sex mismatch; CMV mismatch (D+/R-); ≤ 3 HLA mismatches

* Data presented as mean (SD), median (IQR), and frequency (percentage)

** LAS = lung allocation score; PaO2 = arterial oxygen tension; FiO2 = fraction of inspired oxygen;

CMV = cytomegalovirus; D+ = donor positive; R- = recipient negative; HLA = human leukocyte antigen

 


Disclosure:

E. J. Bernstein,
None;

E. R. Peterson,
None;

J. M. Bathon,
None;

D. J. Lederer,
None.

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