Clinical characteristics and treatment response of patients with SLE complicated with thrombotic thrombocytopenic purpura

作者：Kai Zhang, Peng Zhao, Bo Huang, Yifan Wang, Tianshu Bi, Peiliang Gao, Chunyu Wang, Xiaoyan Xing, Naidi Wang, Ruiling Feng, Gong Cheng, Yao HaiHong, Yuan An, Yunshan Zhou, Yuebo Jin, Yuhui Li, Z. Li, Jing He · 发表于：Lupus Science & Medicine · 年份：2025 · DOI：10.1136/lupus-2025-001740 · 被引用次数：4 · 研究领域：Complement system in diseases、Blood groups and transfusion、Coagulation, Bradykinin, Polyphosphates, and Angioedema

BACKGROUND: SLE complicated with thrombotic thrombocytopenic purpura (SLE-TTP) is a rare but potentially fatal condition. Current studies regarding SLE-TTP are limited to case reports and literature reviews. This study presents a cohort of patients with SLE-TTP and aims to investigate their clinical characteristics and treatment outcomes, as well as to explore the efficacy of rituximab (RTX) maintenance therapy (RMT) for relapse prevention and long-term disease control. METHODS: Patients with SLE-TTP were retrospectively identified in an SLE cohort. Baseline characteristics, acute-phase treatment responses and long-term outcomes were collected. All patients received RTX-containing induction therapy during the acute phase of TTP. Maintenance therapy was categorised as RMT (regular RTX infusions) or non-RMT (conventional immunosuppressants and/or biologics) regimens. TTP relapse, lupus low disease activity state (LLDAS) and infection rates were compared between groups. RESULTS: Of 33 patients with SLE-TTP, 31 (94%) achieved clinical remission following RTX-containing induction therapy, while 2 died during the acute phase. Fourteen patients (45%) received RMT, and 17 (55%) received non-RMT regimens. During a median follow-up of 22.9 months, TTP relapse occurred in seven (23%) patients: one (7%) in the RMT group and six (35%) in the non-RMT group. Kaplan-Meier analysis revealed significantly longer relapse-free survival with RMT (log-rank p=0.027). All patients receiving RMT achi...