A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation |
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Authors: | Kazushi Kinjo Hiroya Terabe Megumi Ogawa Hideki Takeda Doki Cho Shinichi Hoshino Takeshi Miyahira Mariko Oshiro |
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Institution: | (1) Department of Internal Medicine, Okinawa Prefectural Hokubu Hospital, 2-12-3 Onaka, Nago Okinawa, 905-8512, Japan;(2) Department of Pathology, Okinawa Prefectural Hokubu Hospital, 2-12-3 Onaka, Nago Okinawa, 905-8512, Japan |
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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.
This work was performed at Okinawa Prefectural Hokubu Hospital. |
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Keywords: | Acute respiratory distress syndrome Catastrophic antiphospholipid syndrome Multiorgan failure Pulmonary complication |
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