Kai Zhang, Peng Zhao, Bo Huang, Yifan Wang, Taoran Bi, Peiliang Gao, Chunyu Wang, Xiaoyan Xing, Naidi Wang, Ruiling Feng, Gong Cheng, Haihong Yao, Yuan An, Yunshan Zhou, Yuebo Jin, Yuhui Li, Zhanguo Li, Jing He
{"title":"SLE合并血栓性血小板减少性紫癜的临床特点及治疗效果。","authors":"Kai Zhang, Peng Zhao, Bo Huang, Yifan Wang, Taoran Bi, Peiliang Gao, Chunyu Wang, Xiaoyan Xing, Naidi Wang, Ruiling Feng, Gong Cheng, Haihong Yao, Yuan An, Yunshan Zhou, Yuebo Jin, Yuhui Li, Zhanguo Li, Jing He","doi":"10.1136/lupus-2025-001740","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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 achieved LLDAS, compared with 59% of patients in the non-RMT group. Infection rates were comparable between the two groups.</p><p><strong>Conclusions: </strong>RTX-containing induction regimens resulted in high rates of clinical remission in patients with SLE-TTP. RMT was associated with a significantly reduced risk of TTP relapse and superior long-term control of SLE disease activity, without an excess risk of severe infection. These findings support RMT as a potential option for long-term management of SLE-TTP.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 2","pages":""},"PeriodicalIF":3.5000,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical characteristics and treatment response of patients with SLE complicated with thrombotic thrombocytopenic purpura.\",\"authors\":\"Kai Zhang, Peng Zhao, Bo Huang, Yifan Wang, Taoran Bi, Peiliang Gao, Chunyu Wang, Xiaoyan Xing, Naidi Wang, Ruiling Feng, Gong Cheng, Haihong Yao, Yuan An, Yunshan Zhou, Yuebo Jin, Yuhui Li, Zhanguo Li, Jing He\",\"doi\":\"10.1136/lupus-2025-001740\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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 achieved LLDAS, compared with 59% of patients in the non-RMT group. Infection rates were comparable between the two groups.</p><p><strong>Conclusions: </strong>RTX-containing induction regimens resulted in high rates of clinical remission in patients with SLE-TTP. RMT was associated with a significantly reduced risk of TTP relapse and superior long-term control of SLE disease activity, without an excess risk of severe infection. These findings support RMT as a potential option for long-term management of SLE-TTP.</p>\",\"PeriodicalId\":18126,\"journal\":{\"name\":\"Lupus Science & Medicine\",\"volume\":\"12 2\",\"pages\":\"\"},\"PeriodicalIF\":3.5000,\"publicationDate\":\"2025-09-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Lupus Science & Medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1136/lupus-2025-001740\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Lupus Science & Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1136/lupus-2025-001740","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
Clinical characteristics and treatment response of patients with SLE complicated with thrombotic thrombocytopenic purpura.
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 achieved LLDAS, compared with 59% of patients in the non-RMT group. Infection rates were comparable between the two groups.
Conclusions: RTX-containing induction regimens resulted in high rates of clinical remission in patients with SLE-TTP. RMT was associated with a significantly reduced risk of TTP relapse and superior long-term control of SLE disease activity, without an excess risk of severe infection. These findings support RMT as a potential option for long-term management of SLE-TTP.
期刊介绍：
Lupus Science & Medicine is a global, peer reviewed, open access online journal that provides a central point for publication of basic, clinical, translational, and epidemiological studies of all aspects of lupus and related diseases. It is the first lupus-specific open access journal in the world and was developed in response to the need for a barrier-free forum for publication of groundbreaking studies in lupus. The journal publishes research on lupus from fields including, but not limited to: rheumatology, dermatology, nephrology, immunology, pediatrics, cardiology, hepatology, pulmonology, obstetrics and gynecology, and psychiatry.
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