Antiphospholipid antibody syndrome presenting as a refractory noninflammatory pulmonary vasculopathy.
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.
Kerr
J E
JE
Department of Medicine, University of Rochester School of Medicine, Highland Hospital, NY, USA.
Poe
R
R
Kramer
Z
Z
D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis*; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis*; D001706:Biopsy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000150:complications / Q000175:diagnosis*; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000175:diagnosis / Q000209:etiology; D012128:Respiratory Distress Syndrome / Q000175:diagnosis / Q000209:etiology; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis*