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Antiphospholipid Antibody Syndrome Presenting as a Refractory Noninflammatory Pulmonary Vasculopathy

Jamie E. Kerr; Robert Poe; Zachary Kramer
Author and Funding Information

Affiliations: From the Department of Medicine, University of Rochester School of Medicine, Highland Hospital, Rochester, NY.,  From the Departments of Medicine and Oncology, and the Pulmonary Disease Unit, University of Rochester School of Medicine, Highland Hospital, Rochester, NY.

Affiliations: From the Department of Medicine, University of Rochester School of Medicine, Highland Hospital, Rochester, NY.,  From the Departments of Medicine and Oncology, and the Pulmonary Disease Unit, University of Rochester School of Medicine, Highland Hospital, Rochester, NY.


1997 by the American College of Chest Physicians


Chest. 1997;112(6):1707-1710. doi:10.1378/chest.112.6.1707
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Abstract

The clinical manifestations of antiphospholipid antibody syndrome (APLAS) are protean. Pulmonary manifestations are often thromboembolic in origin; ARDS and pulmonary hypertension have been reported as features of a widespread vasculopathy associated with systemic lupus or Sjögren's syndrome. This is the report of a woman with primary APLAS who died of a noninflammatory pulmonary vasculopathy. The case is unusual in its pulmonary manifestations, its initial response to corticosteroids and antithrombotic medications, its failure to stabilize with high-intensity warfarin sodium and aspirin treatment, and finally its fulminant progression despite multiple interventions.


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