Background Catastrophic antiphospholipid syndrome (CAPS) - thrombotic microvasculopathy, characterized by involvement of systems and organs with formation of their insufficiency. Therapy of glucocorticoids and immunosuppressants with plasmapheresis and intravenous immunoglobulin demonstrates lack of effectivity. Currently, there are reports of rituximab effectiveness in APS and CAPS, but they are not numerous, especially CAPS.
Methods Assessment of the rituximab effectiveness in patients with resistant CAPS. We present the treatment results of two patients with CAPS, who were treated in our clinic.
Results Patient V., 21, was admitted in the early postpartum period. Diagnosis: Systemic lupus erythematosus, positivity for ANA: ds-DNA, Sm. Secondary antiphospholipid syndrome (positivity for β2-glycoprotein-1, cardiolipin, lupus anticoagulant), catastrophic APS with multiorgan failure: epilepsy, sopor, psychosis; acute renal injury, signs of acute respiratory distress syndrome and hypocoagulation. SLEDAI score was 56 points, GAPSS – 17 points. Pulse therapy with methylprednisolone, cyclophosphamide, plasmapheresis and intravenous immunoglobulin wasn’t effective enough. Rituximab course 375 mg/m2 1 time per week for 4 weeks was administered. Manifestations of CAPS in multiorgan failure form regressed 14 days after the first administration, SLEDAI score decreased to 32 points, GAPSS to 4 points. After 4 weeks complete B-cell depletion was achieved. A month after the course of rituximab she achieved remission, which lasts 4 years already. ANA and antiphospholipid antibodies weren’t detected.
Patient E., 20, with primary APS, cardiolipin positivity, β2-glycoprotein-1 and lupus anticoagulant, thrombocytopenia, livedo reticularis; CAPS-like thrombotic microangiopathy type with damage to cerebral vessels, lung vessels, recurrent pulmonary embolism for six months, deep leg vein thrombosis. GAPSS activity before treatment was 17 points. Pulse therapy wasn’t performed. Therapy rituximab 375 mg/m2 1 time per week, 4 weeks was administered. Multiple organ failure also regressed 10–14 days after 1 administration of rituximab. GAPSS score decreased to 10 points. Incomplete B-cell depletion was achieved.
Conclusion Thus, rituximab demonstrated high effectiveness in CAPS in both cases. Rituximab allowed to reach multiple organ failure regression and a persistent effect was achieved.
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