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Phone: (843) 805-6930
West Ashley Library
9 a.m. - 4 p.m.
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John L. Dart Library
9 a.m. - 6 p.m.
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St. Paul's/Hollywood Library
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Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature.
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- Author(s): Stanescu, Cristina; Andronesi, Andreea Gabriella; Jurcut, Ciprian; Gherghiceanu, Mihaela; Vornicu, Alexandra; Burcea, Florentina Andreea; Andronesi, Toader Danut; Lupusoru, Gabriela Elena; Iliuta, Luminita; Sorohan, Bogdan Marian; Obrisca, Bogdan; Ismail, Gener
- Source:
Medicina (1010660X); Sep2021, Vol. 57 Issue 9, p1-12, 12p- Subject Terms:
- Source:
- Additional Information
- Abstract: 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’ 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+Blymphocytes (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. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Medicina (1010660X) is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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