• Cristina Stanescu, Andreea Gabriella Andronesi, Ciprian Jurcut, Mihaela Gherghiceanu, Alexandra Vornicu, Florentina Andreea Burcea, Toader Danut Andronesi, Gabriela Elena Lupusoru, Luminita Iliuta, Bogdan Marian Sorohan, Bogdan Obrisca, and Gener Ismail.
• Nephrology Department, Fundeni Clinical Institute, 022328 Bucharest, Romania.
• Medicina (Kaunas). 2021 Aug 31; 57 (9).

BackgroundKidney 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 PresentationWe 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+B-lymphocytes (CD19+B-Ly) after one month. We further review the current knowledge regarding pathogenesis and management of CAPS in SLE patients.ConclusionsTargeted 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.

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