Abstract: Tumor-associated autoantibodies are produced by abnormal exposure or presentation of tumor-associated antigen that promote autoimmune responses, elevation of which could be months or years in advance and participate in the occurrence and development of malignant transformation of tumors. In recent years, clinical application of tumor-associated autoantibodies has become increasingly prominent and provided a reference for early warning, risk assessment, diagnosis, prognosis and therapeutic efficacy in patients with cancer. This review mainly focused on the production mechanism, the status quo and future prospective of clinical application as well as research progress of tumor-associated autoantibodies in connective tissue diseases combined with tumors and malignancy.