Cardiovascular Disease is a Leading Cause of Mortality among TTP Survivors in Clinical Remission.

iTTP survivors experience high rates of adverse health sequelae and increased mortality over long-term follow up. We conducted this multi-center cohort study to evaluate long-term mortality and causes of death in iTTP survivors. Between 2003 and 2020, 222 patients were enrolled in the Ohio State University and Johns Hopkins TTP registries and followed for a median of 4.5 (interquartile range [IQR],75 0.4-11.5) years. Nine patients died during their first iTTP episode and 29 patients died during follow-up. Mortality rate was 1.8 times higher than expected from an age, sex and race adjusted reference population. Cardiovascular disease was a leading primary cause of death (27.6%) tied with relapsed iTTP (27.6%), followed by malignancy (20.7%), infection (13.8%), and other causes (10.3%). Male sex [HR 3.74 (95% CI 1.65-8.48, P=0.002), increasing age [HR 1.04 (95% CI 1.01-1.07), P=0.011] and number of iTTP episodes [HR 1.10 (95% CI 1.01-1.20), P=0.022] were associated with mortality in a model adjusted for African American race [HR 0.70 (95% CI 0.30-1.65), P=0.702], hypertension [HR 0.47 (95% CI 0.20-1.08), P=0.076], CKD [HR 1.46 (95% CI 0.65-3.30, P=0.358] and site [HR 1.46 (95% CI 0.64-3.30), P=0.358]. There was a trend towards shorter survival in patients with lower ADAMTS13 activity during remission (P=0.078). In conclusion, iTTP survivors are at higher risk of death compared with a reference population and cardiovascular disease is a leading cause of death. Our study highlights the need for survivorship care, and investigation focused on cardiovascular disease and early mortality in TTP survivors.