Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations

M. L. Rangel, I. Alghamdi, Gabriel Contreras, Thomas Harrington, David Thomas, Laura Barisoni-Thomas, David Andrews, M. Wolf, A. Asif, Ali Nayer

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)855-864
Number of pages10
JournalLupus
Volume22
Issue number8
DOIs
StatePublished - Jul 1 2013

Fingerprint

Antiphospholipid Syndrome
Hemorrhage
Autoantibodies
Thrombosis
beta 2-Glycoprotein I
Thrombotic Microangiopathies
Complement C4
Kidney
Lupus Coagulation Inhibitor
Systemic Inflammatory Response Syndrome
Lung
Cardiolipins
Plasma Exchange
Liver Function Tests
Antinuclear Antibodies
Gastroenteritis
Prothrombin
Serologic Tests
Hematuria
Platelet Count

Keywords

  • CAPS
  • Catastrophic antiphospholipid syndrome
  • diffuse alveolar hemorrhage
  • glucocorticoids
  • hemorrhage
  • interstitial hemorrhage
  • plasma exchange
  • plasmapheresis
  • thrombotic microangiopathy

ASJC Scopus subject areas

  • Rheumatology

Cite this

Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations. / Rangel, M. L.; Alghamdi, I.; Contreras, Gabriel; Harrington, Thomas; Thomas, David; Barisoni-Thomas, Laura; Andrews, David; Wolf, M.; Asif, A.; Nayer, Ali.

In: Lupus, Vol. 22, No. 8, 01.07.2013, p. 855-864.

Research output: Contribution to journalArticle

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