title: The antiphospholipid syndrome in patients with systemic lupus erythematosus.
abstract: The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction. The management of patients with SLE and APS/aPL should include an accurate stratification of vascular risk factors. Low dose aspirin and hydroxychloroquine should be considered as primary prophylaxis. In high risk situations, such as surgery, prolonged immobilization, and puerperium, the prophylaxis should be potentiated with low molecular weight heparin. The challenge of treating patients with a previous vascular event (secondary prophylaxis) is the choice of treatment (anti-platelet agents, anticoagulation with vitamin K antagonists or combined therapy) and its duration, based on individual risk stratification and the site of vascular presentation. The role of novel anticoagulants in APS patients is still to be clearly defined. Novel approaches are needed since the prognosis of SLE patients with APS/aPL is still worse than that of SLE patients with negative aPL. The goal for the future is to improve the outcome of these patients by means of early recognition and optimal preventative treatment.
authors: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors: Guillermo J
authors: GJ
authors: Pons-Estel
authors: Department of Clinical and Experimental Sciences, University of Brescia, Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy.
authors: Laura
authors: L
authors: Andreoli
authors: Department of Clinical and Experimental Sciences, University of Brescia, Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy.
authors: Francesco
authors: F
authors: Scanzi
authors: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors: Ricard
authors: R
authors: Cervera
authors: Department of Clinical and Experimental Sciences, University of Brescia, Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy. Electronic address: angela.tincani@unibs.it.
authors: Angela
authors: A
authors: Tincani
mesh_terms: D017152:Antibodies, Antiphospholipid / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D001323:Autoantibodies / Q000276:immunology; D003937:Diagnosis, Differential; D019468:Disease Management; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D009026:Mortality; D015995:Prevalence; D063189:Symptom Assessment; D013927:Thrombosis / Q000209:etiology / Q000517:prevention & control / Q000628:therapy
chemical_list: D017152:Antibodies, Antiphospholipid; D001323:Autoantibodies