From the Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing; Thrombosis and Hemostasis Research Unit, Jiangsu Institute of Hematology, First Affiliated Hospital of Soochow University, Suzhou; Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
C. Yue, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; J. Su, MD, Thrombosis and Hemostasis Research Unit, Jiangsu Institute of Hematology, First Affiliated Hospital of Soochow University; R. Gao, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; Y. Wen, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; C. Li, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; G. Chen, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; X. Zhang, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College; X. Li, MD, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College.
J Rheumatol. 2018 Nov;45(11):1549-1556. doi: 10.3899/jrheum.170811. Epub 2018 Jul 15.
To investigate the characteristics and outcomes of patients with systemic lupus erythematosus (SLE)-associated thrombotic microangiopathy (TMA) based on their ADAMTS13 inhibitor profiles.
The medical data of 31 SLE patients with clinically diagnosed TMA were analyzed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients.
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 ± 5.1 vs 25.0 ± 17.8 × 10/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 ± 18.0 vs 21.6 ± 12.0, p < 0.001), lower proteinuria level [0.6 (0.2-2.5) vs 8.1 (5.2-14.0) g/d, p = 0.011], and lower mean arterial pressure (95.3 ± 13.6 vs 117.5 ± 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 ± 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 1.8-65.5, p < 0.001) when ADAMTS13 inhibitor was present in SLE-associated TMA.
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.
基于 ADAMTS13 抑制剂谱，探讨系统性红斑狼疮（SLE）相关血栓性微血管病（TMA）患者的特征和结局。
分析了 31 例临床诊断为 TMA 的 SLE 患者的医学数据。所有患者均检测 ADAMTS13 活性和 ADAMTS13 抑制剂。
TMA 归因于 19 例活动期 SLE。其中 6 例检测到 ADAMTS13 抑制剂和严重 ADAMTS13 缺乏。ADAMTS13 抑制剂组（n=6）的血小板计数（7.3±5.1 vs 25.0±17.8×10/l，p=0.005）和更常见的中枢神经系统（CNS）受累（100.0% vs 23.1%，p=0.003）低于无 ADAMTS13 抑制剂组（n=13）。ADAMTS13 抑制剂组患者的肾脏受累也较轻，表现为估计肾小球滤过率较高（112.7±18.0 vs 21.6±12.0，p<0.001），蛋白尿水平较低[0.6（0.2-2.5）vs 8.1（5.2-14.0）g/d，p=0.011]，平均动脉压较低（95.3±13.6 vs 117.5±13.1mmHg，p=0.008）。所有 ADAMTS13 抑制剂组患者在 18.6±8.7 天内完全缓解，而无 ADAMTS13 抑制剂组患者在中位随访 5.0 个月时，有 3 例（23.1%）患者完全缓解，尽管该组患者接受了更多的治疗性血浆置换（100.0% vs 50.0%，p=0.021）。当 SLE 相关 TMA 存在 ADAMTS13 抑制剂时，完全缓解的机会增加了 10.8 倍（HR 10.8，95%CI 1.8-65.5，p<0.001）。
获得性 ADAMTS13 缺乏与更严重的血小板减少和 CNS 受累、轻度肾脏受累、快速缓解以及相对较好的治疗反应相关在 SLE 相关 TMA 中。
