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Catastrophic APS in the Context of Other Thrombotic Microangiopathies

  • Antiphospholipid Syndrome (D Erkan, Section Editor)
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Abstract

The catastrophic antiphospholipid syndrome (CAPS) is a rare disease that affects 1 % of cases with antiphospholipid syndrome (APS). CAPS can mimic or overlap with different thrombotic diseases; many patients present with a microthrombotic storm or thrombotic microangiopathic hemolytic anemia (TMHA). Thus, the differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP), typical and atypical hemolytic uremic syndrome (HUS), systemic infections, malignancies, pregnancy-related disorders, malignant hypertension, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathies. Antiphospholipid antibody (aPL) positivity has been proposed as the clue in this differential diagnosis; however, aPL can also occur in healthy people and in those with infections or malignancies. Thus, the differential diagnosis of an aPL-positive patient presenting with a microthrombotic storm is broad; the workup should include a special attention to signs of infection and disseminated malignant disease, assessing the funduscopic signs of malignant hypertension, testing ADAMTS13 activity and anti-heparin-platelet factor 4 (HPF4) antibodies, and searching previous exposure to certain drugs. This article aims to review the main diseases included in the differential diagnosis of CAPS in the context of other thrombotic microangiopathies.

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Ignasi Rodríguez-Pintó, Gerard Espinosa, and Ricard Cervera declare no conflicts of interest.

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  1. Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain

    Ignasi Rodríguez-Pintó, Gerard Espinosa & Ricard Cervera

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  1. Ignasi Rodríguez-Pintó
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  2. Gerard Espinosa
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  3. Ricard Cervera
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Correspondence to Ricard Cervera.

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This article is part of the Topical Collection on Antiphospholipid Syndrome

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Rodríguez-Pintó, I., Espinosa, G. & Cervera, R. Catastrophic APS in the Context of Other Thrombotic Microangiopathies. Curr Rheumatol Rep 17, 3 (2015). https://doi.org/10.1007/s11926-014-0482-z

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