{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T04:04:20Z","timestamp":1789185860008,"version":"build-2803163510"},"update-to":[{"DOI":"10.12688\/f1000research.51295.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T00:00:00Z","timestamp":1653436800000}}],"reference-count":11,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T00:00:00Z","timestamp":1653436800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"Thrombotic\u00a0thrombocytopenic\u00a0purpura (TTP) is an\u00a0uncommon\u00a0microangiopathic disease and sometimes is associated with systemic\u00a0lupus\u00a0erythematous (SLE).\u00a0However, this probable causal relationship\u00a0has\u00a0not\u00a0been\u00a0completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a case of a 32-year-old woman with antecedents of epileptic seizures since she was 12 years old. The patient was admitted to the\u00a0emergency\u00a0room with a clinical picture of headaches, fever, paleness in the skin and mucosa,\u00a0confused\u00a0state, paresthesia, and transient spasticity of the extremities. The laboratory\u00a0results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film.\u00a0In addition, positive antinuclear antibodies\u00a0and positive anti-native DNA\u00a0in titers of 1\/320\u00a0and\u00a01\/160, respectively,\u00a0were found.\u00a0Urinalysis showed that serum creatinine was in normal range. Because of limited hospital resources, ADAMTS13 was not evaluated. However, based on clinical, hematological, and biochemical findings, we concluded that it was a case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response\u00a0(normalization of biomarker levels, health condition)\u00a0after the second session of plasmapheresis.\u00a0Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions,\u00a0may\u00a0lead\u00a0to\u00a0good clinical response.<\/ns4:p>","DOI":"10.12688\/f1000research.51295.2","type":"journal-article","created":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T11:45:06Z","timestamp":1653479106000},"page":"552","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura,\u00a0a diagnostic challenge"],"prefix":"10.12688","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9726-4752","authenticated-orcid":false,"given":"Horacio","family":"Su\u00e1rez-Ale","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Fabian-Aquino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2992-9342","authenticated-orcid":false,"given":"Virgilio E.","family":"Failoc-Rojas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9158-1108","authenticated-orcid":false,"given":"Vicente A","family":"Benites-Zapata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9360-8722","authenticated-orcid":false,"given":"Felipe","family":"Ignacio-Cconchoy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2560","published-online":{"date-parts":[[2022,5,25]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"220-226","DOI":"10.1007\/s12254-018-0429-6","article-title":"Thrombotic thrombocytopenic purpura.","volume":"11","author":"P Knobl","year":"2018","journal-title":"Memo."},{"key":"ref2","doi-asserted-by":"publisher","first-page":"824-829","DOI":"10.1093\/rheumatology\/keq395","article-title":"Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.","volume":"50","author":"F Lansigan","year":"2011","journal-title":"Rheumatology (Oxford, England)."},{"key":"ref3","doi-asserted-by":"publisher","first-page":"782-787","DOI":"10.12659\/ajcr.898955","article-title":"Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature.","volume":"17","author":"M Abu-Hishmeh","year":"2016","journal-title":"Am J Case Rep."},{"key":"ref4","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/s13052-019-0641-y","article-title":"Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases.","volume":"45","author":"J Li","year":"2019","journal-title":"Ital J Pediatr."},{"key":"ref5","doi-asserted-by":"publisher","first-page":"357-362","DOI":"10.1038\/ncprheum0511","article-title":"Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties.","volume":"3","author":"A Shah","year":"2007","journal-title":"Nat Clin Pract Rheumatol."},{"key":"ref6","doi-asserted-by":"publisher","first-page":"399-403","DOI":"10.1093\/rheumatology\/ken510","article-title":"A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus.","volume":"48","author":"P Letchumanan","year":"2009","journal-title":"Rheumatology (Oxford, England)."},{"key":"ref7","doi-asserted-by":"publisher","first-page":"419-427","DOI":"10.1007\/s10067-013-2312-5","article-title":"Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011.","volume":"33","author":"H Jiang","year":"2014","journal-title":"Clin Rheumatol"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2014-208477","article-title":"Thrombotic thrombocytopenic purpura as an initial presentation of systemic lupus erythematosus with acquired ADAMTS 13 antibody.","volume":"2015","author":"B Changcharoen","year":"2015","journal-title":"BMJ Case Rep."},{"key":"ref9","doi-asserted-by":"publisher","first-page":"1147-1150","DOI":"10.7534\/j.issn.1009-2137.2017.04.033","article-title":"Clinical Analysis of 12 cases of Systemic Lupus Erythematosus Associated with Thrombotic Thrombocytopenic Purpura.","volume":"25","author":"Y Pan","year":"2017","journal-title":"Zhongguo shi yan xue ye xue za zhi."},{"key":"ref10","doi-asserted-by":"publisher","first-page":"e0256024","DOI":"10.1371\/journal.pone.0256024","article-title":"Outcomes in 1096 patients with severe thrombotic thrombocytopenic purpura before the Caplacizumab era.","volume":"16","author":"A Van de Louw","year":"2021","journal-title":"PLoS One."},{"key":"ref11","doi-asserted-by":"publisher","first-page":"454-458","DOI":"10.1007\/s12026-017-8898-y","article-title":"Thrombotic thrombocytopenic purpura in a new onset lupus patient?","volume":"65","author":"N Gonzalez","year":"2017","journal-title":"Immunol Res."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/10-552\/v2\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/10-552\/v2\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/10-552\/v2\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,12]],"date-time":"2023-07-12T05:15:12Z","timestamp":1689138912000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/10-552\/v2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,25]]},"references-count":11,"URL":"https:\/\/doi.org\/10.12688\/f1000research.51295.2","relation":{"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.134308.r139003","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.134308.r179420","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.134308.r139003","asserted-by":"object"},{"id-type":"doi","id":"10.5256\/f1000research.134308.r179420","asserted-by":"object"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,25]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/10-552\/v2#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.54448.r97915, Katerina Pavenski, Department of Medicine and Laboratory Medicine, St. Michael's Hospital - Unity Health Toronto, Toronto, 15 Nov 2021, version 1, 1 approved with reservations, 1 not approved","URL":"https:\/\/f1000research.com\/articles\/10-552\/v1#referee-response-97915","order":0,"name":"referee-response-97915","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"Virgilio E Failoc-Rojas<\/b>; \nPosted: 21 May 2022<\/i>; \nTitle<\/b> Based on reading this case report, I am not convinced that your patient fulfils the diagnostic criteria for SLE. She clearly has positive autoimmune serology but does not appear to have any clinical features of SLE. Finding of positive autoimmune serology is not uncommon in patients with iTTP, and some of these patients eventually develop SLE and some of them will not. \nAuthor Response:<\/b> In fact, the patient met the criteria for SLE, but we did not detail the clinical manifestations. The patient had a history of epileptic seizures and oral ulcer, and on admission presented with malar erythema. Given the initial suspicion of SLE, subsequent immunological tests confirmed the diagnosis (positive antinuclear antibodies in titers of 1\/320, and positive antinuclear DNA in titers of 38.24 IU\/mL). We have sought to clarify the diagnostic process of SLE in the text. \nAbstract<\/b> 1. Suggest to change "TTP often occurs as a complication of SLE" to "TTP sometimes is associated with SLE". The latter statement is more accurate. \nAuthor Response: <\/b>Thank you. We have changed the statement. 2. Re-word "direct Coombs negative hemolytic anemia". You surely do not mean "autoimmune" hemolytic anemia which is associated with spherocytes on film and RBC autoantibodies. \nAuthor Response: <\/b>We have re-worded the phrase. 3. What is "conserved" renal function? Do you mean her renal function was at baseline on the basis of measuring her serum creatinine, or serum creatinine was in normal range? Was urinalysis done? \nAuthor Response:<\/b> We clarified this term. Yes, urinalysis was done and showed that serum creatinine was in the normal range. 4. Was troponin done to rule out cardiac involvement? \nAuthor Response:<\/b> Yes, troponin was done and ruled out cardiac involvement. 5. ADAMTS13 activity and inhibitor were not done in this case but are required to confirm diagnosis. Please discuss this limitation and way to mitigate it (i.e. in resource constrained circumstance, can use a prediction score - such as PLASMIC) to predict chance of having low ADAMTS13. \nAuthor Response: <\/b>Thank you, this limitation was discussed briefly. \nIntroduction<\/b> 1. The classical pentad occurs in under 10% of cases of TTP. It is therefore important to question TTP when there is MAHA, thrombocytopenia and no obvious explanation and not wait for the pentad. \nAuthor Response:<\/b> Thank you for your comment. We agreed with this idea; this was pointed out in the text. 2. Completely disagree with statement "generally, TTP is a complication in patients with SLE". In most patients, iTTP is not associated with SLE or any other disorder. Indeed, to see SLE and TTP is not common at all. \nAuthor Response:<\/b> Thank you. We clarified this statement, as the message was to explain that TTP might occur after SLE as a complication, in the context of the rare presentation of both entities. \nCase presentation<\/b> 1. What is meant by mixed ancestry? \nAuthor Response:<\/b> This was a term referred to as mestizo. We changed this term for sake of clarity. 2. Does "irregular treatment" with carbamazapine mean inconsistent compliance? \nAuthor Response:<\/b> Yes, it does. To clarify the term, we changed it to \u201chas been treated inconsistently with carbamazepine\u201d. 3. Was there any history of obstetrical complications? Were antecedent births live and healthy? Were there any pregnancy losses? \nAuthor Response: <\/b>The patient had three preterm delivery events. All births were live and healthy and there were no pregnancy losses. This was added in the text. 4. Why were platelets transfused and repeatedly - 5 times? Based on lab work provided, very high chance of this being TTP (for e.g., if use PLASMIC score) and platelet transfusions are considered contra-indicated in patients with TTP (risk of worsening thrombosis) unless there is a life-threatening bleed. Was she seriously bleeding? \nAuthor Response: <\/b>Treatment was defined after consultation with experts based on clinical and platelet levels. We agree that this treatment is not the most appropriate but in the limited circumstances of the hospital it was the most accepted option. 5. Why was plasma exchange not started right away or only after worsening of clinical and labs? Based on presenting labs, very high index of suspicion that this was TTP...why PEX was stopped after 4 treatments? Standard of care is usually daily PEX until platelets are normal for at least 2 consecutive days. \nAuthor Response:<\/b> The reason immediate treatment with plasma exchange was not performed was that TTP was not initially suspected, and the availability of treatment was not immediate to provide timely care. In addition, treatment with PEX was momentarily suspended after four treatments because, by expert consensus, the patient showed a favorable pattern in which the platelet level was increasing, and the DHL concentration was decreasing. Finally, upon seeing a consistent improvement, it was decided not to restart the plasma exchange. This response was also added to the text. \nDiscussion<\/b> 1. I disagree with "TTP does not usually present alone". In fact, in majority of cases, TTP presents alone, with no other diseases. \nAuthor Response:<\/b> Thank you for your comment. We agree with this statement and have therefore revised the term in the text. 2. I understand that there was a diagnostic uncertainty about her initial diagnosis: SLE vs TTP. And that treatment of SLE was appropriately steroid pulse. However, based on her presenting picture (fragmentation hemolysis and thrombocytopenia and evidence of target organ damage - neurological) TTP could not be ruled out and as such (SLE or not) she should have received PEX or risk mortality or further organ damage. For every day delay in starting TPE, risk of death increases (Van de Louw \net al.,<\/i> 2021\n1<\/sup>). This should be the message of this case report. \nAuthor Response:<\/b> Thank you for this insightful comment. We recognize that this was a limitation and agree that PTT should have been appropriately ruled out regardless of the diagnosis of SLE. We have added this message in the text. 3. I also would encourage the authors to include the statement about importance of getting ADAMTS13 activity test. If APLA, other autoimmune sophisticated serology could be done by the lab, so could be ADAMTS13. And this test is very important precisely because it is so difficult to differentiate TTP from other diseases, especially if more than one disease is present. \nAuthor Response: <\/b>Thank you for your kind suggestion. Indeed, ADAMTS13 activity testing is a critical step in the diagnosis of TTP. However, many hospitals in Peru lack these and other sophisticated tests due to cost. With this in mind, we have added a statement on the importance of ADAMTS13 so that it may benefit the proper clinical management of PTT in future events. 4. Re sentence, "TTP should always be considered...however in young women SLE and TTP can occur simultaneously": This is not the reason to delay TTP treatment. TTP is most likely in young women and so is SLE. TTP should always be considered and treatment should be started, whether SLE is also suspected and especially in young women. \nAuthor Response:<\/b> Thank you. The sentence was modified. We agree that in young women with these characteristics, one should think about TTP regardless of concomitant disease, and in the face of this suspicion act in a timely manner. \nConclusion<\/b> Really, conclusion should be that SLE and TTP can occur together (albeit not frequently) and this makes diagnosis more difficult. However, considering the severe consequences of leaving TTP untreated (death, major organ damage), patient should be treated expediently as TTP with PEX and steroids while diagnosis is being sorted out. \nAuthor Response: <\/b>Thank you for your suggestion. We have modified this section to convey a more precise conclusion.","URL":"https:\/\/f1000research.com\/articles\/10-552\/v2#referee-comment-8278","order":1,"name":"referee-comment-8278","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.134308.r139003, Ahmet Emre E\u015fkazan, Division of Hematology, Department of Internal Medicine, Cerrahpa\u015fa Faculty of Medicine, Istanbul University-Cerrahpa\u015fa, \u0130stanbul, 09 Jun 2022, version 2, indexed","URL":"https:\/\/f1000research.com\/articles\/10-552\/v2#referee-response-139003","order":2,"name":"referee-response-139003","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.134308.r179420, Edinson Dante Meregildo-Rodriguez, Escuela de Medicina, Cesar Vallejo University, Trujillo, La Libertad, 12 Jul 2023, version 2, indexed","URL":"https:\/\/f1000research.com\/articles\/10-552\/v2#referee-response-179420","order":3,"name":"referee-response-179420","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The author(s) declared that no grants were involved in supporting this work","order":4,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}