BOSTON -- Patients with systemic sclerosis (SSc) who have end-stage lung disease can benefit from lung transplantation despite having higher 1-year mortality rates compared with patients with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) not related to SSc, a researcher reported here.
For patients with SSc, there was a 48% increase in 1-year mortality risk (HR 1.48, 95% CI 1.01-2.17) following lung transplantation compared with non-SSc ILD, reported , of Columbia University in New York City.
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- Note that these studies were published as abstracts and presented at a conference. These data and conclusions should be considered to be preliminary until published in a peer-reviewed journal.
But no increase in 1-year mortality was seen for patients post-transplant who had non-SSc PAH (HR 0.85, 95% CI 0.50-1.44).
"The first lung transplant was done in 1963, and the patient survived for only 18 days. Today the median survival is 5.7 years, and in 2013 there were 1,923 lung transplants in the U.S.," Bernstein said during a plenary session at the
Only a few small series of lung transplantation in SSc have been reported, and in these, survival was similar to that of ILD and PAH, which are accepted indications for the procedure.
"Lung transplantation is a potentially lifesaving treatment for patients with systemic sclerosis who have developed end-stage lung disease. However, many transplant programs are hesitant to offer lung transplantation to those with SSc due to concerns about extra-pulmonary involvement that might affect short- and long-term survival," Bernstein said.
To see if those concerns were valid, she conducted a retrospective cohort study of adults who underwent either single or double lung transplantation between May 2005, when the lung allocation scoring system was implemented, and Sept. 2012.
The data were obtained from the United Network for Organ Sharing, which collects data on all U.S. solid organ transplants.
For inclusion in the study, patients had to have been at least 18 years old and to have had a diagnosis of SSc, ILD, or PAH.
Patients were excluded if they had been given a lung transplant from a living donor or if they had a heart-lung transplant.
The cohort included 3,763 patients. Of these, 229 had SSc, 201 had PAH, and 3,333 had ILD.
Mean ages were 53 in the SSc group, 46 in the PAH group, and 62 in the ILD group. Women made up 59% and 63% of the SSc and PAH groups, but only 28% of the ILD group.
Pulmonary artery systolic pressures were 48, 83, and 39 mm Hg in the three groups, respectively, and forced vital capacities were 44%, 73%, and 45% of predicted.
Mechanical ventilation had been needed in 10.04%, 2.99%, and 6.96%, while oxygen requirements were 5, 4, and 4 L/min, respectively.
A total of 52% of patients with SSc were on steroids, and more of the SSc group required extracorporeal membrane oxygenation and mechanical ventilation than in the other two groups.
The unadjusted 1-year mortality rates were 21.4 per 100 person-years in the SSc group, 19 per 100 in the PAH group, and 17.8 per 100 in the ILD group.
The 48% increased mortality rate for patients with SSc was seen after adjustment for multiple factors, including age, sex, race, use of steroids, smoking history, pulmonary function tests, and recipient-donor sex and HLA mismatches.
Bernstein also looked at 3-year mortality, and found rates of 7.9 per 100 person-years for SSc, 12.6 per 100 for PAH, and 12 per 100 for ILD.
"Rather than denying SSc patients lung transplantation because of their diagnosis, variables need to be identified that will enable risk stratification of patients prior to transplantation, with particular attention to modifiable risk factors. A prospective study should consider this," she concluded.
Disclosures
Bernstein had no disclosures.