A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation

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 char...

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Bibliographic Details
Published in:	Rheumatology international Vol. 29; no. 2; pp. 211 - 216
Main Authors:	Kinjo, Kazushi, Terabe, Hiroya, Ogawa, Megumi, Takeda, Hideki, Cho, Doki, Hoshino, Shinichi, Miyahira, Takeshi, Oshiro, Mariko
Format:	Journal Article
Language:	English
Published:	Berlin/Heidelberg Springer-Verlag 01-12-2008 Springer Nature B.V
Subjects:	Antiphospholipid Syndrome - complications Antiphospholipid Syndrome - diagnosis Antiphospholipid Syndrome - physiopathology Case Report Diagnosis, Differential Fatal Outcome Female Humans Kidney - pathology Medical diagnosis Medicine Medicine & Public Health Middle Aged Multiple Organ Failure - etiology Multiple Organ Failure - pathology Multiple Organ Failure - physiopathology Respiratory distress syndrome Respiratory Distress Syndrome - complications Respiratory Distress Syndrome - diagnosis Respiratory Distress Syndrome - physiopathology Respiratory Insufficiency - etiology Respiratory Insufficiency - pathology Respiratory Insufficiency - physiopathology Rheumatology Acute respiratory distress syndrome Pulmonary complication Catastrophic antiphospholipid syndrome Multiorgan failure
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Summary:	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.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	0172-8172 1437-160X
DOI:	10.1007/s00296-008-0649-x