ADAMTS13 Activity Stratifies SLE-Associated Thrombotic Microangiopathy: Rituximab versus Cyclophosphamide in Severe Deficiency Subgroup: A Retrospective Cohort Study
- 1Department of Rheumatology and Immunology, The Second Affiliated Hospital of Xiamen Medical College, Xiamen, 361021, People’s Republic of China
Abstract
Background/Objectives: Systemic lupus erythematosus (SLE)-associated thrombotic microangiopathy (TMA) is a severe complication with high mortality. This study aimed to examine clinical manifestations, treatment strategies, and outcomes in these patients.
Methods: This retrospective cohort study included 64 patients diagnosed with SLE-associated TMA between January 2016 and April 2025. Patients were stratified by ADAMTS13 activity into SLE-associated immune-mediated thrombotic thrombocytopenic purpura (SLE-iTTP, ADAMTS13 <10%) and non-iTTP TMA (ADAMTS13 ≥10%) groups. Clinical characteristics, laboratory findings, treatment responses, and outcomes were compared between the groups. Within the SLE-iTTP subgroup, outcomes were compared between patients receiving rituximab (RTX) and those receiving cyclophosphamide (CYC) as adjunctive immunosuppressive therapy.
Results: Among 64 patients, 36 (56.3%) were classified as SLE-iTTP and 28 (43.7%) as non-iTTP TMA. Patients with SLE-iTTP exhibited more severe thrombocytopenia (median platelet count: 5.5 vs 39 × 109/L, p<0.001), lower hemoglobin levels (49.5 vs 69 g/L, p<0.001), and a higher frequency of neuropsychiatric manifestations (88.9% vs 57.1%, p=0.008). Anti-dsDNA positivity (86.1% vs 57.1%, p=0.012) and hypocomplementemia (94.4% vs 71.4%, p=0.016) were more prevalent in the SLE-iTTP group. Within the SLE-iTTP subgroup, all patients received glucocorticoids and plasma exchange. Patients treated with RTX (n=20) achieved a significantly higher rate of clinical remission or response than those treated with CYC (n=16) (85.0% vs 50.0%, p=0.034) and showed faster platelet count normalization (median 14 vs 21 days, p=0.018). Overall mortality was 34.4% (22/64).
Conclusion: ADAMTS13 activity identifies a distinct subset of SLE-associated TMA with characteristic features and differential treatment responses. In SLE-iTTP, RTX-based regimens was associated with better clinical efficacy than CYC, supporting a pathophysiology-guided therapeutic approach for this life-threatening condition.
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BibTeX
@article{huang2026adamts13,
title = {{ADAMTS13 Activity Stratifies SLE-Associated Thrombotic Microangiopathy: Rituximab versus Cyclophosphamide in Severe Deficiency Subgroup: A Retrospective Cohort Study}},
author = {Jinmei Huang and Wei Fan and Pingping Xiao and Shufan Wu and Yi Zhang and Hua Li and Xuyan Chen and Zhigao Dong},
journal = {International Journal of General Medicine},
year = {2026},
month = oct,
doi = {10.2147/ijgm.s653266},
url = {https://doi.org/10.2147/ijgm.s653266},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - ADAMTS13 Activity Stratifies SLE-Associated Thrombotic Microangiopathy: Rituximab versus Cyclophosphamide in Severe Deficiency Subgroup: A Retrospective Cohort Study
AU - Jinmei Huang
AU - Wei Fan
AU - Pingping Xiao
AU - Shufan Wu
AU - Yi Zhang
AU - Hua Li
AU - Xuyan Chen
AU - Zhigao Dong
PY - 2026
DA - 2026/10/30
JO - International Journal of General Medicine
DO - 10.2147/ijgm.s653266
UR - https://doi.org/10.2147/ijgm.s653266
AB - Background/Objectives: Systemic lupus erythematosus (SLE)-associated thrombotic microangiopathy (TMA) is a severe complication with high mortality. This study aimed to examine clinical manifestations, treatment strategies, and outcomes in these patients. Methods: This retrospective cohort study included 64 patients diagnosed with SLE-associated TMA between January 2016 and April 2025. Patients were stratified by ADAMTS13 activity into SLE-associated immune-mediated thrombotic thrombocytopenic purpura (SLE-iTTP, ADAMTS13 <10%) and non-iTTP TMA (ADAMTS13 ≥10%) groups. Clinical characteristics, laboratory findings, treatment responses, and outcomes were compared between the groups. Within the SLE-iTTP subgroup, outcomes were compared between patients receiving rituximab (RTX) and those receiving cyclophosphamide (CYC) as adjunctive immunosuppressive therapy. Results: Among 64 patients, 36 (56.3%) were classified as SLE-iTTP and 28 (43.7%) as non-iTTP TMA. Patients with SLE-iTTP exhibited more severe thrombocytopenia (median platelet count: 5.5 vs 39 × 109/L, p<0.001), lower hemoglobin levels (49.5 vs 69 g/L, p<0.001), and a higher frequency of neuropsychiatric manifestations (88.9% vs 57.1%, p=0.008). Anti-dsDNA positivity (86.1% vs 57.1%, p=0.012) and hypocomplementemia (94.4% vs 71.4%, p=0.016) were more prevalent in the SLE-iTTP group. Within the SLE-iTTP subgroup, all patients received glucocorticoids and plasma exchange. Patients treated with RTX (n=20) achieved a significantly higher rate of clinical remission or response than those treated with CYC (n=16) (85.0% vs 50.0%, p=0.034) and showed faster platelet count normalization (median 14 vs 21 days, p=0.018). Overall mortality was 34.4% (22/64). Conclusion: ADAMTS13 activity identifies a distinct subset of SLE-associated TMA with characteristic features and differential treatment responses. In SLE-iTTP, RTX-based regimens was associated with better clinical efficacy than CYC, supporting a pathophysiology-guided therapeutic approach for this life-threatening condition.
SN - 1178-7074
ER -