Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.

Rangel, ML

Alghamdi, I

Contreras, G

Harrington, T

Thomas, DB

Barisoni, L

Andrews, D

Wolf, M

Asif, A

Nayer, A

2021-04-01T15:02:52Z

2021-04-01T15:02:52Z

2013-07

2021-04-01T15:02:52Z

Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.

0961203313491024

0961-2033

1477-0962

https://hdl.handle.net/10161/22498

eng

SAGE Publications

Lupus

10.1177/0961203313491024

Pulmonary Alveoli

Humans

Respiratory Insufficiency

Thrombosis

Antiphospholipid Syndrome

Catastrophic Illness

Hemorrhage

Antibodies, Antinuclear

Glucocorticoids

Plasma Exchange

Dose-Response Relationship, Drug

Adult

Female

Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.

Journal article

Barisoni, L|0000-0003-0848-9683

Wolf, M|0000-0002-1127-1442

855

864

8

School of Medicine

Pathology

Medicine, Nephrology

Duke

Clinical Science Departments

Medicine

Duke Clinical Research Institute

Population Health Sciences

Institutes and Centers

Basic Science Departments

Published

22