The ASTIS trial (Autologous Stem cell Transplantation International Scleroderma), launched in 2001, evaluated the efficacy of autologous haematopoietic stem cell transplantation (HSCT) in patients with systemic sclerosis. A key point to safe use of HSCT is a correct evaluation of cardiac condition, and a close follow up for cardiac complications. In a letter to the Editor published in The Journal of the American Medical Association, entitled “Cardiac Assessment Before Stem Cell Transplantation for Systemic Sclerosis,” Dr. Burt at the Division of Immunotherapy, Northwestern University and colleagues highlight the importance of performing extensive cardiopulmonary screening in patients with severe forms of systemic sclerosis before administrating HSCT.
HSCT therapy first involves harvesting patients’ stem cells. Since Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease (autoimmune diseases are characterized by a hyper-reactive response of the immune response against substances, and tissues normally present in the body), the second step of the process involves destroying patients’ hyper-reactive immune system using chemotherapy. Afterward, the patients’ harvested stem cells will be injected back into the body. The objective is to reset the patient immune system to normal standards and thus stop the process of scleroderma.
Systemic sclerosis is associated with many cardiac complications, including intrinsic myocardial ischemia and fibrosis, left ventricular diastolic dysfunction, and pericardial disease. While the criteria for exclusion in the ASTIS trial was mean pulmonary artery pressure greater than 50 mm Hg by echo-cardiogram or cardiac catheterization, the authors emphasize that this does not exclude pulmonary arterial hypertension. Despite the fact that 2009 guidelines updated their information and described pulmonary arterial hypertension as a mean pulmonary artery pressure higher than 25 mm Hg, the authors cautioned that a significant amount of attention has to be dedicated to assessing cardiac risks in these patients to prevent treatment-related mortality.