{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T23:38:29Z","timestamp":1783640309952,"version":"3.55.0"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T00:00:00Z","timestamp":1659312000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Thrombotic microangiopathy (TMA) is a syndrome associated with hemolytic anemia, thrombocytopenia, and various organ disorders. Thrombotic thrombocytopenic purpura (TTP) is a disease that develops when a disintegrin-like and metalloproteinase with thrombospondin type l motif 13 (ADAMTS13) activity decreases to &amp;lt; 10% of that in normal plasma, causing platelet thrombosis in microvessels throughout the body. Currently, ADAMTS13-deficient TMA is diagnosed as TTP. Systemic lupus erythematosus (SLE)-related TMA includes both acquired TTP, in which ADAMTS13 activity is significantly reduced, and secondary TMA, in which ADAMTS13 activity is not reduced. Both diseases have different prognoses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>An 11-year-old girl was admitted to our hospital on suspicion of TMA with thrombocytopenia and hemolytic anemia. Because the patient had hypocomplementemia, SLE-related TMA or complement-related TMA was considered. Therefore, we initiated plasma exchange (PE) for the patient. Subsequently, she fulfilled the pediatric SLE diagnostic criteria, and ADAMTS13 activity was shown to be decreased and the anti-ADAMTS13 antibody titer increased. She was thus diagnosed with acquired TTP caused by SLE. Treatment response was good as a platelet count and ADAMTS13 activity improved with three times of PE, followed by methylprednisolone pulse therapy and administration of mycophenolate mofetil. Renal pathology showed thrombus formation in glomerular arterioles and lupus nephritis categorized as Class III (A) of the International Society of Nephrology and the Renal Pathology Society classification. Because the patient was thought to be in the high-risk group of SLE, three courses of intravenous cyclophosphamide pulse therapy were administered as an additional induction therapy. No recurrence of TTP was observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In SLE-related TMA, measurement of ADAMTS13 activity and the anti-ADAMTS13 antibody titer are necessary for diagnosis, and for predicting prognosis and recurrence of the disease; however, in the acute phase of immune-mediated TMA, it is important to initiate proper treatments even before knowing the results to improve prognosis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2022.931669","type":"journal-article","created":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T05:36:36Z","timestamp":1659332196000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report"],"prefix":"10.3389","volume":"10","author":[{"given":"Yoko","family":"Takagi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuko","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayako","family":"Hirakata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariko","family":"Takei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Ogasawara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chikage","family":"Yajima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuka","family":"Ikeuchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Matsumoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiyuki","family":"Ogawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Handa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Matsumoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirokazu","family":"Arakawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takumi","family":"Takizawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,8,1]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1847","DOI":"10.1056\/NEJMc1410951","article-title":"Syndromes of thrombotic microangiopathy.","volume":"371","author":"George","year":"2014","journal-title":"N Engl J Med."},{"key":"B2","doi-asserted-by":"publisher","first-page":"7","DOI":"10.2169\/internalmedicine.49.2706","article-title":"Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.","volume":"49","author":"Fujimura","year":"2010","journal-title":"Intern Med."},{"key":"B3","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1007\/s12185-017-2264-7","article-title":"Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan.","volume":"106","author":"Matsumoto","year":"2017","journal-title":"Int J Hematol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"1549","DOI":"10.3899\/jrheum.170811","article-title":"Characteristics and outcomes of patients with systemic lupus erythematosus-associated thrombotic microangiopathy, and their acquired ADAMTS13 inhibitor profiles.","volume":"45","author":"Yue","year":"2018","journal-title":"J Rheumatol."},{"key":"B5","first-page":"739","article-title":"Thrombotic microangiopathies.","author":"Laszik","year":"2015","journal-title":"Heptinstall\u2019s Pathology of the Kidney."},{"key":"B6","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1007\/s002770050184","article-title":"Von Willebrand factor: molecular size and functional activity.","volume":"72","author":"Furlan","year":"1996","journal-title":"Ann Hematol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1016\/S0092-8674(00)80983-","article-title":"Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor.","volume":"84","author":"Savage","year":"1996","journal-title":"Cell."},{"key":"B8","first-page":"454","article-title":"Analyses of ADAMTS13 activity and its inhibitor in patients with thrombotic thrombocytopenic purpura secondary to connective tissue diseases: observations in a single hospital.","volume":"24","author":"Sato","year":"2006","journal-title":"Clin Exp Rheumatol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1676","DOI":"10.1002\/pbc.24612","article-title":"Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features.","volume":"60","author":"Reese","year":"2013","journal-title":"Pediatr Blood Cancer."},{"key":"B10","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1160\/TH08-12-0825","article-title":"Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.","volume":"102","author":"Matsuyama","year":"2009","journal-title":"Thromb Haemost."},{"key":"B11","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1056\/NEJM199108083250604","article-title":"Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian apheresis study group.","volume":"325","author":"Rock","year":"1991","journal-title":"N Engl J Med."},{"key":"B12","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1007\/BF01696619","article-title":"Thrombotic thrombocytopenic purpura\/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange.","volume":"70","author":"Pereira","year":"1995","journal-title":"Ann Hematol."},{"key":"B13","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1007\/s00277-009-0877-5","article-title":"High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study.","volume":"89","author":"Balduini","year":"2010","journal-title":"Ann Hematol."}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.931669\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T05:36:40Z","timestamp":1659332200000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.931669\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,1]]},"references-count":13,"alternative-id":["10.3389\/fped.2022.931669"],"URL":"https:\/\/doi.org\/10.3389\/fped.2022.931669","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,1]]},"article-number":"931669"}}