title: Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed.
abstract: Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a rare and life-threatening condition that can be easily mistaken with hemolysis elevated liver enzymes low platelets syndrome, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. In order to make a diagnosis, it is required to have multi-organ thrombosis over 1 week affecting at least three organs or systems, and to have positive antiphospholipid antibody on two occasions (6 weeks apart), and histopathologic confirmation of small vessel occlusion. However, due to similarities in clinical and laboratory findings between CAPS and some other obstetric complications, potential misdiagnosis or delay in diagnosis are common, increasing the risk of adverse maternal and perinatal outcomes. In this review we summarized information presented in previous studies, focusing on CAPS related to pregnancy. We reviewed diagnostic criteria, differential diagnosis, and common presentation ranging from malaise, abdominal pain, dyspnea, hypertension, to altered mental status and seizures. We also discussed management in pregnancy and included a detailed algorithm with steps to take. Of note, the most significant reduction in mortality was seen in patients receiving triple therapy which will be discussed in this review.
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Jennifer G
authors.initials: JG
authors.lastname: Hoayek
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Hind N
authors.initials: HN
authors.lastname: Moussa
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Hina A
authors.initials: HA
authors.lastname: Rehman
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Susan Hosseini
authors.initials: SH
authors.lastname: Nasab
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Sean C
authors.initials: SC
authors.lastname: Blackwell
authors.affiliation: a Department of Obstetrics , Gynecology & Reproductive Sciences, University of Texas Health Science Center at Houston , Houston , TX , USA.
authors.forename: Baha M
authors.initials: BM
authors.lastname: Sibai
mesh_terms: D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009102:Multiple Organ Failure; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis*; D011256:Pregnancy Outcome
keywords: AFLP; HELLP; antiphospholipid antibody; antiphospholipid syndrome; imitators of preeclampsia; organ thrombosis