1. A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation
- Author
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Hiroya Terabe, Kazushi Kinjo, Mariko Oshiro, Takeshi Miyahira, Hideki Takeda, Doki Cho, Megumi Ogawa, and Shinichi Hoshino
- Subjects
medicine.medical_specialty ,ARDS ,Multiple Organ Failure ,Immunology ,Acute respiratory distress ,Kidney ,Catastrophic antiphospholipid syndrome ,Diagnosis, Differential ,Fatal Outcome ,Rheumatology ,Antiphospholipid syndrome ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,Intensive care medicine ,Respiratory Distress Syndrome ,business.industry ,Pulmonary Complication ,Middle Aged ,Antiphospholipid Syndrome ,medicine.disease ,Venous thrombosis ,Respiratory failure ,Female ,Respiratory Insufficiency ,business - 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.
- Published
- 2008
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