Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse.
The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS. A detailed description of the clinical and laboratory features of the syndrome can be found in the other articles of this issue.
Bucciarelli
Silvia
S
Department of Autoimmune Diseases, Hospital Clínic, Catalonia, Spain.
Erkan
Doruk
D
Espinosa
Gerard
G
Cervera
Ricard
R
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000367:Age Factors; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D001424:Bacterial Infections / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007165:Immunosuppression Therapy; D008180:Lupus Erythematosus, Systemic / Q000276:immunology*; D009102:Multiple Organ Failure; D010956:Plasmapheresis; D011379:Prognosis; D012008:Recurrence; D012307:Risk Factors; D000069283:Rituximab; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D000069283:Rituximab