Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrosp
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(2020) 15:225
RESEARCH
Open Access
Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study Cai Yue1, Jian Su2, Xiaohong Fan1, Li Song3, Wei Jiang4, Jinghua Xia1, Tao Shi1, Xuan Zhang5,6* and Xuemei Li1*
Abstract Background: Thrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not routinely assayed in most previous studies. The objective of this study is to compare the characteristics and outcomes of immune-mediated TTP (iTTP) in patients with and without SLE. Methods: The medical data of 28 patients with iTTP from Peking Union Medical College Hospital were analysed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients. Results: All 28 patients had ADAMTS13 inhibitor and severe ADAMTS13 deficiency. iTTP was considered SLE-related (SLE-TTP) in 10 patients and primary (primary iTTP) in 18 patients. Renal involvement on presentation was more severe in patients with primary iTTP as determined by higher serum creatinine (162.7 ± 110.6 vs 73.3 ± 13.4 μmol/L, p < 0.01) and more prevalent acute kidney injury (72.2% vs 10.0%, p < 0.01) than in patients with SLE-TTP. More patients with SLE-TTP were treated with steroid pulse therapy (90.0% vs 16.7%, p < 0.01) and intravenous immunoglobulin (IVIG) (50.0% vs 5.6%, p = 0.01) compared to patients with primary iTTP. After adjustments for age and treatment, including steroid pulse therapy and IVIG treatment, the likelihood of clinical remission of SLE-TTP was significantly increased compared to that of primary iTTP (HR 7.6 [1.2, 50.1], p = 0.03). Mortality was also lower among patients with SLE-TTP than among patients with primary iTTP (0 vs 38.9%, p = 0.03). (Continued on next page)
* Correspondence: [email protected]; [email protected] 5 Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, 1 Shuai Fu Yuan, Beijing 100005, China 1 Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, 1 Shuai Fu Yuan, Beijing 100005, China Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the artic
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