J Nephropathol. 2014;3(1): 9-17.
doi: 10.12860/jnp.2014.03
PMID: 24644537
PMCID: PMC3956908
Scopus ID: 84893138466
  Abstract View: 5805
  PDF Download: 2393

Review Article

Catastrophic antiphospholipid syndrome: a clinical review

Ali Nayer 1 * , Luis M. Ortega 2

1 Division of Nephrology and Hypertension, University of Miami, Miami, FL, USA
2 Division of Nephrology and Hypertension, Allegheny General Hospital, Temple University School of Medicine, Pittsburg, PA, USA
Corresponding author: Ali Nayer, MD, Division of Nephrology, University of Miami, Clinical Research Building, Suite 825,1120 NW 14th St., Miami, FL 33136, USA. Email: ANayer@med.miami.edu ANayer@med.miami.edu


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.

Keywords: Catastrophic antiphospholipid, syndrome, Antiphospholipid antibodies, Thrombotic microangiopathy

Implication for health policy/practice/research/medical education:

Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening autoimmune disease characterized by disseminated intravascular thrombosis resulting in multi-organ failure.

Please cite this paper as: Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014; 3(1):9-17. DOI: 10.12860/jnp.2014.03

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ePublished: 01 Jan 2014
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