{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:43:25Z","timestamp":1792590205700,"version":"4.5.0"},"reference-count":31,"publisher":"The Journal of Rheumatology","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>To investigate the characteristics and outcomes of patients with systemic lupus erythematosus (SLE)\u2013associated thrombotic microangiopathy (TMA) based on their ADAMTS13 inhibitor profiles.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>The medical data of 31 SLE patients with clinically diagnosed TMA were analyzed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>TMA was attributable to active SLE in 19 patients. ADAMTS13 inhibitor and severe ADAMTS13 deficiency were detected in 6 of them. Patients with ADAMTS13 inhibitor (n = 6) exhibited a lower platelet count (7.3 \u00b1 5.1 vs 25.0 \u00b1 17.8 \u00d7 10<jats:sup>9<\/jats:sup>\/l, p = 0.005) and more prevalent central nervous system (CNS) involvement (100.0% vs 23.1%, p = 0.003) than patients without ADAMTS13 inhibitor (n = 13). Patients with ADAMTS13 inhibitor also had mild renal involvement characterized by a higher estimated glomerular filtration rate (112.7 \u00b1 18.0 vs 21.6 \u00b1 12.0, p &lt; 0.001), lower proteinuria level [0.6 (0.2\u20132.5) vs 8.1 (5.2\u201314.0) g\/d, p = 0.011], and lower mean arterial pressure (95.3 \u00b1 13.6 vs 117.5 \u00b1 13.1 mmHg, p = 0.008) than was observed in patients without ADAMTS13 inhibitor. All patients with ADAMTS13 inhibitor achieved complete remission within 18.6 \u00b1 8.7 days, while 3 patients (23.1%) without ADAMTS13 inhibitor achieved complete remission during a median followup of 5.0 months, even though more patients in this group received therapeutic apheresis (100.0% vs 50.0%, p = 0.021). The chance of complete remission increased by 10.8-fold (HR 10.8, 95% CI 1.8\u201365.5, p &lt; 0.001) when ADAMTS13 inhibitor was present in SLE-associated TMA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>Acquired ADAMTS13 deficiency is associated with more severe thrombocytopenia and CNS involvement, mild renal involvement, rapid resolution, and relatively good treatment response in SLE-associated TMA.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.170811","type":"journal-article","created":{"date-parts":[[2018,7,15]],"date-time":"2018-07-15T07:25:11Z","timestamp":1531639511000},"page":"1549-1556","source":"Crossref","is-referenced-by-count":25,"title":["Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus\u2013associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor 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