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Autoimmun Rev. 2015 May;14(5):401-14. doi: 10.1016/j.autrev.2015.01.002. Epub 2015 Jan 29.

The relevance of "non-criteria" clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features.

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Disciplina de Reumatologia, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. Electronic address:
Serviço de Reumatologia, Hospital do Servidor Estadual do Rio de Janeiro, Rio de Janeiro, Brazil.
Vascular Medicine Division and Regional Competence Centre For Rare Vascular and Systemic Autoimmune Diseases, Centre Hospitalier Universitaire (CHU), Nancy, France; Institut Lorrain du Coeur et des Vaisseaux Louis Mathieu, Vandoeuvre-les-Nancy, France; Institut National de la Santé et de la Recherche Médicale (INSERM), U1116, Vandoeuvre-les-Nancy Cedex, France.
ABC Medical Center, México City, Mexico.
First Department of Internal Medicine, University of Athens, Athens, Greece.
Pedro Ernesto University Hospital of the Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY, USA.
Centro di Ricerche di Immunopatologia e Documentazione su Malattie Rare and Università di Torino, Dipartimento di Scienze Cliniche e Biologiche, Torino, Italy; Graham Hughes Lupus Research Laboratory, King's College London, London, UK.
Rio de Janeiro Municipality, Rio de Janeiro, Brazil.
Johns Hopkins University School of Medicine, Baltimore, USA.
Lupus Research Unit at St Thomas' Hospital, London, UK.
Discipline of Rheumatology, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.


The purpose of this task force was to critically analyze nine non-criteria manifestations of APS to support their inclusion as APS classification criteria. The Task Force Members selected the non-criteria clinical manifestations according to their clinical relevance, that is, the patient-important outcome from clinician perspective. They included superficial vein thrombosis, thrombocytopenia, renal microangiopathy, heart valve disease, livedo reticularis, migraine, chorea, seizures and myelitis, which were reviewed by this International Task Force collaboration, in addition to the seronegative APS (SN-APS). GRADE system was used to evaluate the quality of evidence of medical literature of each selected item. This critical appraisal exercise aimed to support the debate regarding the clinical picture of APS. We found that the overall GRADE analysis was very low for migraine and seizures, low for superficial venous thrombosis, thrombocytopenia, chorea, longitudinal myelitis and the so-called seronegative APS and moderate for APS nephropathy, heart valve lesions and livedo reticularis. The next step can be a critical redefinition of an APS gold standard, for instance derived from the APS ACTION registry that will include not only current APS patients but also those with antiphospholipid antibodies not meeting current classification criteria.


Accuracy; Antiphospholipid Syndrome; Clinical features; GRADE system; Non-criteria manifestations

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