Catastrophic antiphospholipid syndrome: A clinical review

Ali Nayer, Luis M. Ortega

Research output: Contribution to journalArticle

48 Citations (Scopus)

Abstract

Context: Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening autoimmune disease characterized by disseminated intravascular thrombosis resulting in multiorgan failure. Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, PubMed (NLM), LISTA (EBSCO) and Web of Science have been searched. Results: CAPS is due to antiphospholipid antibodies directed against a heterogeneous group of proteins that are associated with phospholipids. These autoantibodies activate endothelial cells, platelets, and immune cells, thereby promoting a proinflammatory and prothrombotic phenotype. Furthermore, antiphospholipid antibodies inhibit anticoagulants, impair fibrinolysis, and activate complements. Although CAPS can affect a variety of organs and tissues, the kidneys, lungs, central nervous system, heart, skin, liver, and gastrointestinal tract are most commonly affected. The systemic inflammatory response syndrome, likely to extensive tissue damage, accompanies CAPS. The most frequent renal manifestations are hypertension, proteinuria, hematuria, and acute renal failure. In the majority of patients with CAPS, a precipitating factor such as infection, surgery, or medication can be identified. Antiphospholipid antibodies such as lupus anticoagulant and antibodies against cardiolipin, β2-glycoprotein I, and prothrombin are serological hallmark of CAPS. Laboratory tests often reveal antinuclear antibodies, thrombocytopenia, and anemia. Despite widespread intravascular coagulation, blood films reveal only a small number of schistocytes. In addition, severe thrombocytopenia is uncommon. Conclusions: Histologically, CAPS is characterized by acute thrombotic microangiopathy. CAPS must be distinguished from other forms of thrombotic microangiopathies such as hemolytic-uremic syndrome, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, and heparin-induced thrombocytopenia. CAPS is associated with high morbidity and mortality. Therefore, an aggressive multidisciplinary treatment strategy is indicated. Anticoagulation, immunosuppression, plasma exchange, intravenous immunoglobulins, and anti-platelet agents, used in various combinations, have resulted in improved patient outcome.

Original languageEnglish (US)
Pages (from-to)9-17
Number of pages9
JournalJournal of Nephropathology
Volume3
Issue number1
DOIs
StatePublished - 2014

Fingerprint

Antiphospholipid Syndrome
Antiphospholipid Antibodies
Thrombocytopenia
Thrombotic Microangiopathies
Blood Platelets
Thrombotic Thrombocytopenic Purpura
Directories
Precipitating Factors
Lupus Coagulation Inhibitor
Systemic Inflammatory Response Syndrome
Hemolytic-Uremic Syndrome
Cardiolipins
Renal Hypertension
Plasma Exchange
Disseminated Intravascular Coagulation
Antinuclear Antibodies
Intravenous Immunoglobulins
Prothrombin
Fibrinolysis
Hematuria

Keywords

  • Antiphospholipid antibodies
  • Catastrophic antiphospholipid
  • Syndrome
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Nephrology

Cite this

Catastrophic antiphospholipid syndrome : A clinical review. / Nayer, Ali; Ortega, Luis M.

In: Journal of Nephropathology, Vol. 3, No. 1, 2014, p. 9-17.

Research output: Contribution to journalArticle

Nayer, Ali ; Ortega, Luis M. / Catastrophic antiphospholipid syndrome : A clinical review. In: Journal of Nephropathology. 2014 ; Vol. 3, No. 1. pp. 9-17.
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