title: Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient.
abstract: Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.
authors.affiliation: Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan.
authors.forename: Masanobu
authors.initials: M
authors.lastname: Horikoshi
authors.forename: Shigeko
authors.initials: S
authors.lastname: Inokuma
authors.forename: Erika
authors.initials: E
authors.lastname: Matsubara
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Honda
authors.forename: Rika
authors.initials: R
authors.lastname: Okada
authors.forename: Mika
authors.initials: M
authors.lastname: Kobuna
authors.forename: Yoko
authors.initials: Y
authors.lastname: Miura
authors.forename: Shoko
authors.initials: S
authors.lastname: Kobayashi
authors.forename: Shigeo
authors.initials: S
authors.lastname: Kawai
authors.forename: Toshio
authors.initials: T
authors.lastname: Kumasaka
mesh_terms: D000368:Aged; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000473:pathology*; D001344:Autopsy; D002388:Catastrophic Illness; D002761:Cholangitis / Q000209:etiology / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D009102:Multiple Organ Failure / Q000188:drug therapy / Q000473:pathology*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy / Q000473:pathology*; D012008:Recurrence; D000069283:Rituximab / Q000627:therapeutic use*
chemical_list: D005938:Glucocorticoids; D000069283:Rituximab