Objective To investigate the clinical characteristics of patients with systemic vasculitis in China by analyzing the data from a nationwide registry database, the Chinese Registry for Systemic Vasculitis (CRSV). Methods The demographic data, clinical presentations, image and laboratory test results, disease activity assessment, treatment, and outcome of patients enrolled with the confirmed diagnosis of systemic vasculitis, including Takayasu’s arteritis (TAK), ANCA-associated vasculitis (AAV), Behcet’s syndrome (BD), Polyangitis nodosa (PAN), and unclassified systemic vasculitis (USV), were collected since July 2013 in the CRSV. The data during July 2013 and February 28 of 2020 were retrieved and analyzed. Results Up to February 2020, 3852 patients in total were registered in the CRSV. In Peking Union Medical College Hospital (PUMCH), 1348 patients were registered, including 730 patients with TAK, 343 with Behcet’s disease (BD), 191 with AAV, 53 with USV, and 31 with PAN. More male patients were found in PAN (64.5%), BD (59.8%), and AAV (52.4%), while TAK was more prevalent in female patients (85.9%). The average age at disease diagnosis was 29.6 in TAK, 33.2 in BD, 28.6 in PAN, 39.4 in USV, and 48.1 in AAV. A significant difference was found between age at diagnosis of TAK and AAV. Gender comparisons revealed that the age at diagnosis of male patients with AAV was older than female patients. Hypertension (57.3%) and stroke (17.5%) were found to be more common in male patients with TAK, vascular involvement (27.3%) was found to be more common in male patients with BD, while neurological involvement (29.0%) was found to be more common in male patients with AAV than in females. Conclusion This registry is the first in China and provides rich information about the clinical characteristics of Chinese patients with systemic vasculitis. There are significant differences in clinical presentations between different categories of systemic vasculitis.