title: [Catastrophic antiphospholipid syndrome].
abstract: The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition resulting from rapidly progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. Within a few days, the patients develop multiorgan failure with pulmonary distress, renal failure with severe hypertension, cerebral, cardiac, digestive or cutaneous involvement. CAPS develops in less than 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. CAPS may be precipitated by infectious diseases, surgical procedures or discontinuation of anticoagulation. CAPS overall mortality rate has decreased in the past decade and is now around 30%. Within our hospital, it has been reduced to 10%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the empirical association of anticoagulation and corticosteroids, plus plasma exchange or intravenous immunoglobulins. Cyclophosphamide is added in patients with systemic lupus erythematosus. The prevention of CAPS is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment of infections and the education of patients with antiphospholipid syndrome.
authors.affiliation: Service de médecine interne, centre de référence national pour le lupus systémique et le syndrome des antiphospholipides, faculté Paris 6, hôpital Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France. nathalie.costedoat@gmail.com
authors.forename: N
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: L
authors.initials: L
authors.lastname: Arnaud
authors.forename: D
authors.initials: D
authors.lastname: Saadoun
authors.forename: J
authors.initials: J
authors.lastname: Chastre
authors.forename: G
authors.initials: G
authors.lastname: Leroux
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
authors.forename: Z
authors.initials: Z
authors.lastname: Amoura
authors.forename: J-C
authors.initials: JC
authors.lastname: Piette
mesh_terms: D017152:Antibodies, Antiphospholipid / Q000097:blood; D000925:Anticoagulants / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D015415:Biomarkers / Q000097:blood; D002388:Catastrophic Illness / Q000628:therapy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000628:therapy; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D009102:Multiple Organ Failure / Q000209:etiology / Q000628:therapy; D010951:Plasma Exchange; D051437:Renal Insufficiency / Q000209:etiology / Q000628:therapy; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D015415:Biomarkers; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors