ArticleOrphanet journal of rare diseases2020
Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 19 citations in OpenAlex.
- Clinical predictors of mortality in immune thrombotic thrombocytopenic purpura: A National Inpatient Sample analysis.British journal of haematology · 2026Article
- In reply: epidemiology of immune-mediated thrombotic thrombocytopenic purpura in Germany.Research and practice in thrombosis and haemostasis · 2026Article
- Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome.Lupus science & medicine · 2025Observational
- Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy.Kidney international reports · 2025Article
- Systemic Lupus Erythematosus-Associated Thrombotic Thrombocytopenic Purpura: A Case Report.Cureus · 2025Article
- A peculiar case report of primary iTTP in a previously healthy Saudi man.Journal of medicine and life · 2025Article
- ADAMTS13 Improves Hepatic Platelet Accumulation in Pyrrolizidine Alkaloids-induced Liver Injury.Journal of clinical and translational hepatology · 2025Article
- [Relapsed immune thrombotic thrombocytopenic purpura secondary to systemic lupus erythematosus: a case report and literature review].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2024Review
- Article
- Incidence and medical costs of lupus in Spanish hospitals: a retrospective database analysis.Orphanet journal of rare diseases · 2024Article
- Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura (TTP) With Caplacizumab: A Case Report.Cureus · 2023Article
- Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus.Clinical nephrology. Case studies · 2023Article
- Response to belimumab in thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a case-based review.Clinical rheumatology · 2022Review
- The Efficacy and Safety of Plasma Exchange in the Treatment of Thrombotic Thrombocytopenic Purpura.Journal of healthcare engineering · 2022Article
- Quiescent Systemic Lupus Erythematosus Manifesting with Thrombotic Thrombocytopenic Purpura and Acute Renal Failure: A Case Report and Short Literature Review.Mediterranean journal of rheumatology · 2021Article
- Autoimmune-mediated renal disease and hypertension.Clinical science (London, England : 1979) · 2021Review
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThrombotic 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.
methodsThe 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.
resultsAll 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).
conclusionsRenal involvement was less severe in patients with SLE-TTP than in patients with primary iTTP. The treatment responses and outcomes of SLE-TTP were no worse and perhaps even better than those of primary iTTP. When TTP is diagnosed in SLE patients, the ADAMTS13 level and ADAMTS13 inhibitor profile should be considered in addition to clinical features.
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