{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T00:11:38Z","timestamp":1782951098862,"version":"3.54.5"},"reference-count":49,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2021,8,31]],"date-time":"2021-08-31T00:00:00Z","timestamp":1630368000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background: Kidney involvement is a frequent complication of systemic lupus erythematosus (SLE) and kidney biopsy is essential in differentiating lupus nephritis (LN) from thrombotic microangiopathy (TMA) secondary to antiphospholipid autoantibodies (aPL). Association between antiphospholipid syndrome (APS) and acquired hemophilia due to inhibitors was very rarely described in SLE patients. Case presentation: We present the case of a 61-year-old male diagnosed with SLE who acquired deficiency of clotting factor VIII due to circulating inhibitors, admitted for acute kidney injury (AKI), microangiopathic hemolytic anemia, thrombocytopenia, and diplopia. Kidney biopsy showed TMA due to APS, but no signs of LN. Head computed tomography identified low dense areas in the white matter, suggesting small blood vessels\u2019 involvement. A diagnosis of probable catastrophic antiphospholipid syndrome (CAPS) was established and treatment with low molecular weight heparin, intravenous methylprednisolone, plasmapheresis, and rituximab was initiated, followed by resolution of AKI, diplopia, and TMA with complete depletion of CD19+B-lymphocytes (CD19+B-Ly) after one month. We further review the current knowledge regarding pathogenesis and management of CAPS in SLE patients. Conclusions: Targeted therapy was possible after kidney biopsy, improving renal and general prognosis. CD19+B-Ly repopulation preceded biological relapse, so monitoring of CD19+B-Ly may serve as a tool to predict relapses and guide rituximab therapy.<\/jats:p>","DOI":"10.3390\/medicina57090912","type":"journal-article","created":{"date-parts":[[2021,8,31]],"date-time":"2021-08-31T04:07:28Z","timestamp":1630382848000},"page":"912","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature"],"prefix":"10.3390","volume":"57","author":[{"given":"Cristina","family":"Stanescu","sequence":"first","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5590-7879","authenticated-orcid":false,"given":"Andreea Gabriella","family":"Andronesi","sequence":"additional","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Nephrology Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ciprian","family":"Jurcut","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, \u201cCarol Davila\u201d Military Emergency Hospital, 010225 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mihaela","family":"Gherghiceanu","sequence":"additional","affiliation":[{"name":"\u201cVictor Babes\u201d National Institute for Research and Development in Pathology and Biomedical Sciences, 050097 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Vornicu","sequence":"additional","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florentina Andreea","family":"Burcea","sequence":"additional","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toader Danut","family":"Andronesi","sequence":"additional","affiliation":[{"name":"Department of General Surgery and Liver Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela Elena","family":"Lupusoru","sequence":"additional","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Nephrology Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1439-5002","authenticated-orcid":false,"given":"Luminita","family":"Iliuta","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Marketing and Medical Technology, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3574-4635","authenticated-orcid":false,"given":"Bogdan Marian","family":"Sorohan","sequence":"additional","affiliation":[{"name":"Nephrology Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4796-4950","authenticated-orcid":false,"given":"Bogdan","family":"Obrisca","sequence":"additional","affiliation":[{"name":"Nephrology Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gener","family":"Ismail","sequence":"additional","affiliation":[{"name":"Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Nephrology Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050471 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,31]]},"reference":[{"key":"ref_1","first-page":"17","article-title":"Epidemiology of the Antiphospholipid Syndrome","volume":"Volume 12","author":"Cervera","year":"2016","journal-title":"Handbook of Systemic Autoimmune Diseases"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1097\/BOR.0000000000000269","article-title":"Treatment of catastrophic antiphospholipid syndrome","volume":"28","author":"Kazzaz","year":"2016","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1016\/j.autrev.2016.09.010","article-title":"Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry","volume":"15","author":"Moitinho","year":"2016","journal-title":"Autoimmun. Rev."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1186\/s13059-017-1207-1","article-title":"The MHC locus and genetic susceptibility to autoimmune and infectious diseases","volume":"18","author":"Matzaraki","year":"2017","journal-title":"Genome Biol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1097\/MNH.0b013e3283378f25","article-title":"Thrombotic microangiopathy: New insights","volume":"19","author":"Benz","year":"2010","journal-title":"Curr. Opin. Nephrol. Hypertens."},{"key":"ref_6","first-page":"9","article-title":"Catastrophic antiphospholipid syndrome: A clinical review","volume":"3","author":"Nayer","year":"2014","journal-title":"J. Nephropathol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1182\/blood.2019003863","article-title":"Complement activity and complement regulatory gene mutations are associated with thrombosis in APS and CAPS","volume":"135","author":"Chaturvedi","year":"2020","journal-title":"Blood"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaut.2018.05.007","article-title":"The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review","volume":"92","author":"Cervera","year":"2018","journal-title":"J. Autoimmun."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1016\/j.autrev.2017.10.014","article-title":"Genetic risk factors in thrombotic primary antiphospholipid syndrome: A systematic review with bioinformatic analyses","volume":"17","author":"Islam","year":"2018","journal-title":"Autoimmun. Rev."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"102352","DOI":"10.1016\/j.autrev.2019.102352","article-title":"Antiphospholipid syndrome\u2019s genetic and epigenetic aspects","volume":"18","author":"Iuliano","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1177\/096120330000900109","article-title":"HLA class II alleles associations of anticardiolipin and anti-beta2GPI antibodies in a large series of European patients with systemic lupus erythematosus","volume":"9","author":"Sebastiani","year":"2000","journal-title":"Lupus"},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Rymarz, A., and Niemczyk, S. (2018). The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement. BMC Nephrol., 19.","DOI":"10.1186\/s12882-018-0928-z"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1681\/ASN.V103507","article-title":"The intrarenal vascular lesions associated with primary antiphospholipid syndrome","volume":"10","author":"Nochy","year":"1999","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1111\/j.1440-1797.2007.00792.x","article-title":"Prevalence and clinicopathologic findings of antiphospholipid syndrome nephropathy in Thai systemic lupus erythematosus patients who underwent renal biopsies","volume":"12","author":"Cheunsuchon","year":"2007","journal-title":"Nephrology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2120","DOI":"10.1002\/art.21157","article-title":"Requirement of activation of complement C3 and C5 for antiphospholipid antibody-mediated thrombophilia","volume":"52","author":"Pierangeli","year":"2005","journal-title":"Arthritis Rheum."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1681\/ASN.V94590","article-title":"CD59 protects glomerular endothelial cells from immune-mediated thrombotic microangiopathy in rats","volume":"9","author":"Nangaku","year":"1998","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_17","first-page":"2817","article-title":"Primary antiphospholipid syndrome: Functional outcome after 10 years","volume":"27","author":"Erkan","year":"2000","journal-title":"J. Rheumatol."},{"key":"ref_18","first-page":"1181","article-title":"Renal involvement in primary antiphospholipid syndrome","volume":"19","author":"Amigo","year":"1992","journal-title":"J. Rheumatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1111\/j.1399-0012.2012.01625.x","article-title":"Ten-yr renal allograft survival of patients with antiphospholipid antibody syndrome","volume":"26","author":"Vaidya","year":"2012","journal-title":"Clin. Transplant."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"2051","DOI":"10.1111\/j.1600-6143.2010.03233.x","article-title":"Severe vascular lesions and poor functional outcome in kidney transplant recipients with lupus anticoagulant antibodies","volume":"10","author":"Canaud","year":"2010","journal-title":"Am. J. Transplant."},{"key":"ref_21","first-page":"93","article-title":"Recurrent renal thrombotic angiopathy after kidney transplantation in two patients with primary antiphospholipid syndrome (PAPS)","volume":"8","author":"Bochicchio","year":"1994","journal-title":"Clin. Transplant."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1177\/0961203314538687","article-title":"Identification and treatment of APS renal involvement","volume":"23","author":"Tektonidou","year":"2014","journal-title":"Lupus"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1177\/0961203309361353","article-title":"CAPS Registry Project Group. Catastrophic antiphospholipid syndrome (CAPS): Update from the \u2018CAPS Registry\u2019","volume":"19","author":"Cervera","year":"2010","journal-title":"Lupus"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.berh.2016.07.004","article-title":"Catastrophic antiphospholipid syndrome: The current management approach","volume":"30","author":"Espinosa","year":"2016","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"2568","DOI":"10.1002\/art.22018","article-title":"European Forum on Antiphospholipid Antibodies. Mortality in the catastrophic antiphospholipid syndrome: Causes of death and prognostic factors in a series of 250 patients","volume":"54","author":"Bucciarelli","year":"2006","journal-title":"Arthritis Rheum."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1136\/annrheumdis-2020-218272","article-title":"Update \u03bfn the diagnosis and management of systemic lupus erythematosus","volume":"80","author":"Fanouriakis","year":"2021","journal-title":"Ann. Rheum. Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"195","DOI":"10.22543\/7674.52.P195201","article-title":"New oral anticoagulants and their reversal agents","volume":"5","author":"Laslo","year":"2018","journal-title":"J. Mind Med. Sci."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1656","DOI":"10.1111\/jth.14192","article-title":"McMaster RARE-Best practices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome","volume":"16","author":"Legault","year":"2018","journal-title":"J. Thromb. Haemost."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1146\/annurev-med-042915-102529","article-title":"Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease","volume":"68","author":"Unlu","year":"2017","journal-title":"Annu. Rev. Med."},{"key":"ref_30","first-page":"2006","article-title":"Treatment of catastrophic antiphospholipid syndrome with defibrotide, a proposed vascular endothelial cell modulator","volume":"29","author":"Erkan","year":"2002","journal-title":"J. Rheumatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1097\/00005792-200111000-00002","article-title":"Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients","volume":"80","author":"Asherson","year":"2001","journal-title":"Medicine"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"622","DOI":"10.3389\/fimmu.2018.00622","article-title":"B Cell Modulation Strategies in Autoimmune Diseases: New Concepts","volume":"9","author":"Musette","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"2859","DOI":"10.1038\/jid.2008.178","article-title":"B-cell depletion immunotherapy in pemphigus: Effects on cellular and humoral immune responses","volume":"128","author":"Mouquet","year":"2008","journal-title":"J. Investig. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1002\/art.37759","article-title":"A pilot open label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome","volume":"65","author":"Erkan","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1136\/ard.2007.079418","article-title":"B cell depletion therapy in systemic lupus erythematosus: Relationships among serum B lymphocyte stimulator levels, autoantibody profile and clinical response","volume":"67","author":"Cambridge","year":"2008","journal-title":"Ann. Rheum. Dis."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1016\/j.autrev.2013.05.004","article-title":"Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum on Antiphospholipid Antibodies). Rituximab use in the catastrophic antiphospholipid syndrome: Descriptive analysis of the CAPS registry patients receiving rituximab","volume":"12","author":"Berman","year":"2013","journal-title":"Autoimmun. Rev."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1759720X20910855","DOI":"10.1177\/1759720X20910855","article-title":"An update on the management of antiphospholipid syndrome","volume":"12","author":"Rodziewicz","year":"2020","journal-title":"Ther. Adv. Musculoskelet. Dis."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/cei.12481","article-title":"Usefulness of monitoring of B cell depletion in rituximab-treated rheumatoid arthritis patients in order to predict clinical relapse: A prospective observational study","volume":"180","author":"Trouvin","year":"2015","journal-title":"Clin. Exp. Immunol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1007\/s00415-018-9092-4","article-title":"Peripheral CD19+ B-cell counts and infusion intervals as a surrogate for long-term B-cell depleting therapy in multiple sclerosis and neuromyelitis optica\/neuromyelitis optica spectrum disorders","volume":"266","author":"Ellrichmann","year":"2019","journal-title":"J. Neurol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"359","DOI":"10.34067\/KID.0000072020","article-title":"Clinical response and pattern of B cell suppression with single low dose rituximab in nephrology","volume":"1","author":"Jacob","year":"2020","journal-title":"Kidney360"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.1111\/ajt.12319","article-title":"Eculizumab improves posttransplant thrombotic microangiopathy due to antiphospholipid syndrome recurrence but fails to prevent chronic vascular changes","volume":"13","author":"Canaud","year":"2013","journal-title":"Am. J. Transplant."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4274\/tjh.2015.0197","article-title":"The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome","volume":"33","author":"Erkan","year":"2016","journal-title":"Turk. J. Haematol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2719","DOI":"10.1002\/art.34440","article-title":"Brief report: Induction of sustained remission in recurrent catastrophic antiphospholipid syndrome via inhibition of terminal complement with eculizumab","volume":"64","author":"Shapira","year":"2012","journal-title":"Arthritis Rheum."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1111\/ajt.12540","article-title":"Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation","volume":"14","author":"Lonze","year":"2014","journal-title":"Am. J. Transplant."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"e143","DOI":"10.1097\/MD.0000000000000143","article-title":"Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: A case report","volume":"93","author":"Kronbichler","year":"2014","journal-title":"Medicine"},{"key":"ref_46","unstructured":"ClinicaTrials.gov (2020, September 25). NCT01029587: Eculizumab to Enable Renal Transplantation in Patients with History of Catastrophic Antiphospholipid Antibody Syndrome, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT01029587."},{"key":"ref_47","unstructured":"ClinicaTrials.gov (2020, September 25). NCT02128269: Phase IIa Trial of ALXN1007 for the Treatment of Non-criteria Manifestations of Antiphospholipid Syndrome, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT02128269."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1182\/hematology.2019000073","article-title":"Is there a role for immunosuppression in antiphospholipid syndrome?","volume":"2019","author":"Sevim","year":"2019","journal-title":"Hematol. Am. Soc. Hematol. Educ. Program"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1191\/0961203303lu463cr","article-title":"Coexistence of lupus anticoagulant and acquired haemophilia in a patient with monoclonal gammopathy of unknown significance","volume":"12","author":"Taher","year":"2003","journal-title":"Lupus"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/57\/9\/912\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:52:56Z","timestamp":1760165576000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/57\/9\/912"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,31]]},"references-count":49,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2021,9]]}},"alternative-id":["medicina57090912"],"URL":"https:\/\/doi.org\/10.3390\/medicina57090912","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,31]]}}}