Cause of death in Chinese Takayasu arteritis patients

To analyze the causes of death and the related risk factors for in-patients with Takayasu arteritis (TAK) admitted to a referral center of China during 1983 to 2014.The medical charts of 12 deceased TAK patients (10 women, 2 men) were reviewed by two senior rheumatologists. The demographic data, clinical manifestations, angiographic presentations, and the direct causes of death were analyzed retrospectively. Medical records of 40 TAK patients (32 women, 8 men) were selected as controls by age and sex matching method from 81 patients who were sampled isometrically from 810 successively admitted TAK in-patients of the same center during the same period. In addition to the comparison of clinical manifestations between the two groups, binary logistic regression was conducted to explore the related risk factors of mortality of TAK.Twelve patients died at the median age of 33.5 (ranging from 13 to 68 years old). The median survival time was 102.5(ranging from 6 to 567) months. The direct causes of death were heart failure in 5 (5/12, 41.7%), hemorrhage in 2 (2/12, 16.7%), pulmonary infection in 2 (2/12, 16.7%), sudden death in 1 (1/12, 8.3%), postoperative complication in 1 (1/12, 8.3%), and end-stage malignancy in 1 (1/12, 8.3%). Ischemia (4/12, 33.3%) and hemorrhage (4/12, 33.3%) were the two most common presentations in deceased patients. Eight patients had received surgical procedures related to TAK changes. Among them, 2 patients died after surgical procedure, the other 6 patients died later of non-operation-related causes. Compared with the control group (n = 40), patients in the deceased group had longer disease duration (P = 0.017), higher proportion of active disease (P = 0.020), secondary hypertension (P = 0.004), and congestive heart failure (P = 0.017). A model of binary logistic regression had revealed that secondary hypertension (odds ratio [OR] = 9.333, 95% confidence interval [CI]: 1.721 - 50.614, P = 0.010), congestive heart failure (OR = 5.667, 95% CI: 1.248 - 25.734, P = 0.025), and longer disease duration (OR = 1.007, 95% CI: 1.001 - 0.735, P = 0.027) were risk factors for TAK mortality. Active disease (OR = 0.167, 95% CI: 0.038 - 50.614, P = 0.018) was negatively associated with death of TAK.Heart failure is the leading cause of death in TAK patients, followed by ischemia and pulmonary infection. Early deaths occur postoperatively but become rare later after the procedure. Well-control of hypertension, and prevention of congestive heart failure may improve the long-term prognosis.