Study Urges New Standards For Systemic Sclerosis Lung Transplants

New research presented at the American College of Rheumatology Annual Meeting in Boston highlights that patients with systemic sclerosis with end-stage lung disease cannot be denied lung transplantation on the premises that other extra-pulmonary factors will determine their long-term survival.

Scleroderma, also known as systemic sclerosis (SSc), is an autoimmune disease characterized by skin thickening, a process known as fibrosis. While in some types, this hardening is confined to skin on head, face, and feet, in more severe cases, it affects internal organs such as kidneys, heart, lungs, and intestine. The disease currently lacks an approved treatment and is associated with high mortality rates.

Hospitals can deny lung transplantation to systemic sclerosis patients due to the role of extra-pulmonary factors in determining their survival. Now, researchers at the Columbia University College of Physicians and Surgeons in New York City assessed their one-year mortality rate after lung transplantation in comparison to other autoimmune diseases that are not related with Ss, including interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). They performed a retrospective study with 3,763 adults who received lung transplants (double or single lung transplantation) – 229 with SSc, 201 with PAH and 3,333 with ILD.

They found that while SSc patients had a 48 percent increase in the one-year mortality rate when compared to ILD patients, no increase was observed when compared to PAH patients. Thus, SSc diagnosis cannot solely be the cause for denying lung transplantation, and so the authors urge that other causes need to be identified to perform a stratification of patients in conditions for lung transplant.

Elana J. Bernstein, MD, MS of Columbia University College of Physicians and Surgeons, and a lead author of the study noted, “We found that adults with SSc undergoing lung transplantation in the United States during the lung allocation score era had a 48 percent relative increase in the risk of death at one year compared to those with ILD. In contrast, the risk of death at one year among those with SSc was comparable to those with PAH, a widely accepted indication for lung transplantation. In additional analyses among one-year survivors, we did not detect an increased risk of three-year mortality among those with SSc compared to those with ILD, and found that adults with SSc actually had a lower risk of death at three years than those with PAH. 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.”