Lung Transplantation Decisions For Scleroderma Patients Should Be Rethought, Study Says

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by Isaura Santos |

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New research presented at the American College of Rheumatology’s Annual Meeting in Boston claims that end-stage lung disease patients with diseases like Scleroderma should not be denied the opportunity of receiving a lung transplant because of extra-pulmonary factors and, consequently, short- and long-term survival concerns. The study comes from Researchers at Columbia University College of Physicians and Surgeons in New York City.

Systemic sclerosis (SSc), commonly known as scleroderma, is a rheumatic autoimmune disease that affects the skin and other organs, due to abnormal function of the immune system. SSc is associated with tightening and thickening of the skin and inflammation and scarring of organs that ultimately leads to serious health problems associated with the lungs, heart, kidneys and intestinal system. The condition is rare: only 75,000 to 100,000 people have the disease in the United States, 75 percent of which are women.

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Hospitals are reluctant when it comes to approving lung transplantation in systemic sclerosis patients because of the concerns regarding extra-pulmonary implications that are currently believed to affect patients’ short- and long-term survival.

The research group was determined “to find out if adults with systemic sclerosis have a higher 1-year mortality rate after lung transplantation compared to patients with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) not due to SSc,” as noted in a recent press release. They analyzed 3,763 adults that had suffered double or single lung transplantation between May 4, 2005 and September 14, 2012 in the United States: 229 with SSc, 201 with PAH, and 3,333 with ILD.

The lead author of the study, Elana J. Bernstein, MD, MSc of Columbia University College of Physicians and Surgeons, explained: “Although many transplant centers are hesitant to offer lung transplantation to patients with SSc due to concern about extra-pulmonary involvement that might affect survival, there are very few data to support the impression that survival is worse in SSc patients compared to non-SSc patients (…) In fact, previous studies — albeit limited to small case series and small cohort studies — suggest that adults with SSc have similar mortality rates to adults with ILD and PAH following lung transplantation. We actually hoped to confirm the findings of these prior small studies, thereby perhaps providing an impetus for transplant programs to begin to change their practices and offer lung transplantation to greater numbers of carefully selected patients with SSc who are otherwise suitable candidates for lung transplantation.”

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The results show that the diagnosis of SSc was in fact associated with a 48-percent relative increase in the 1-year death rate compared to a diagnosis of ILD. Nevertheless, a SSc diagnosis was not associated with an increase in the 1-year death rate compared to a diagnosis of PAH, a widely accepted standard for lung transplantation.

In conclusion, researchers said that other variables need to be studied before denying a lung transplantation to SSc patients.

“Future work should aim to prospectively study adults with SSc as they are evaluated for lung transplantation in order to identify potentially modifiable risk factors that can improve transplant outcomes in this population,” said Dr. Bernstein.