title: A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation.
abstract: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss, accompanied by elevated titers of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome (CAPS) is a small subset of APS characterized by widespread systemic thrombotic disease with multiorgan failure. We herein describe an autopsy case of CAPS who developed severe respiratory failure due to acute respiratory distress syndrome (ARDS) as the initial manifestation. Patients with APS may exhibit a broad spectrum of pulmonary diseases. ARDS is the common pulmonary complication in CAPS, although it rarely occurs in APS. Some mechanisms of ARDS in CAPS have been postulated but the precise mechanism is still not clearly understood. It is important to understand that APS or CAPS could be a cause of ARDS since ARDS might develop as the initial manifestation of APS or CAPS as seen in our case. Our case is interesting in that severe respiratory failure due to ARDS was the initial presentation of CAPS.
authors.affiliation: Department of Internal Medicine, Okinawa Prefectural Hokubu Hospital, 2-12-3 Onaka, Nago, Okinawa, 905-8512, Japan. keikinjo@yahoo.co.jp
authors.forename: Kazushi
authors.initials: K
authors.lastname: Kinjo
authors.forename: Hiroya
authors.initials: H
authors.lastname: Terabe
authors.forename: Megumi
authors.initials: M
authors.lastname: Ogawa
authors.forename: Hideki
authors.initials: H
authors.lastname: Takeda
authors.forename: Doki
authors.initials: D
authors.lastname: Cho
authors.forename: Shinichi
authors.initials: S
authors.lastname: Hoshino
authors.forename: Takeshi
authors.initials: T
authors.lastname: Miyahira
authors.forename: Mariko
authors.initials: M
authors.lastname: Oshiro
mesh_terms: D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012128:Respiratory Distress Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology