Catastrophic antiphospholipid syndrome treated with rituximab: A case report

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rare and fatal condition that is characterized by diffuse venous and/or arterial thromboembolism within a short period of time and histopathological confirmation of small-vessel occlusion in at least one organ or tissue in the presence of positive antiphospholipid antibodies. Here we report the case of a 19-year-old woman with CAPS. During the first week of her hospitalization, she was diagnosed with CAPS on the basis of skin necrosis, pulmonary artery thrombosis, cerebral venous sinus thrombosis, and positive lupus anticoagulant. She was treated with corticosteroids, intravenous immunoglobulins, plasmapheresis, and anticoagulants. Forty days after the onset of CAPS, cutaneous lesions were recurred during skin surgery. She required a high dose of corticosteroids, intravenous immunoglobulins, and rituximab. No further thrombotic events occurred. Rituximab may be an effective treatment option for patients with CAPS.

Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage

“…However, the number of patients taking rituximab was too low to determine its effectiveness or safety profile 13. It is believed that rituximab reduces cytokine production by reducing B cells and minimises the systemic inflammatory response 14. Despite treatment with high dose steroid, intravenous immunoglobulin, plasma exchange and immunomodulators like rituximab, CAPS has shown to have high mortality that approaches 50%.…”

Immunosuppressive Treatment in Antiphospholipid Syndrome: Is It Worth It?

“…Rituximab has also been used to treat CAPS with variable response [ 10 , 88 , 89 , 90 , 91 , 119 , 120 ]. For example, Rubenstein et al [ 89 ] reported a patient with CAPS resistant to treatment with heparin and methylprednisolone 1 mg/kg/day, successfully treated with rituximab 375 mg/m 2 once a week for four weeks [ 89 ].…”

Prospects for Anti-B-Cell Therapy in Immuno-Inflammatory Rheumatic Diseases

“…Среди 20 пациентов, получавших лечение РТМ, выздоровление отмечено у 16 (80%), однако 4 пациента умерли. Сходные данные об эффективности РТМ у пациентов с катастрофическим АФС (первичном или в сочетании с СКВ) приводят другие исследователи [131,148,149,[194][195][196].…”