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      Epidemiology of Antiphospholipid Syndrome in the General Population

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          Abstract

          Purpose of Review

          The epidemiology of antiphospholipid syndrome (APS) is poorly understood. Here, we review the current understanding of the epidemiology of antiphospholipid syndrome in the general population and the frequency of antiphospholipid antibodies in the general population in patients with obstetric morbidity, arterial events, and venous thromboembolism.

          Recent Findings

          There have been few population-based studies that estimated the prevalence and incidence of APS. The estimated incidence and prevalence among most these studies ranged between 1 and 2 cases per 100,000 and 40 and 50 cases per 100,000 respectively. The prevalence of antiphospholipid antibodies in patients with obstetric morbidity was 6–9%, while in arterial events and venous thromboembolism is 9–10%. However, this data remains limited. Mortality of patients with APS is 50–80% higher than the general population.

          Summary

          The epidemiology of APS has been difficult to elucidate. Population-based studies patients with diverse age, racial, and ethnic backgrounds are needed.

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          Most cited references55

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          International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).

          S Miyakis, M Lockshin, T Atsumi … (2006)
          New clinical, laboratory and experimental insights, since the 1999 publication of the Sapporo preliminary classification criteria for antiphospholipid syndrome (APS), had been addressed at a workshop in Sydney, Australia, before the Eleventh International Congress on antiphospholipid antibodies. In this document, we appraise the existing evidence on clinical and laboratory features of APS addressed during the forum. Based on this, we propose amendments to the Sapporo criteria. We also provide definitions on features of APS that were not included in the updated criteria.
          Article 0 comments Cited 738 times – based on 0 reviews     Review now
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            Lupus anticoagulant is frequent in patients with Covid‐19

            Inès Harzallah, Agathe Debliquis, Bernard Drénou (2020)
            Patients hospitalized for Covid‐19 severe infection are more prone to excessive coagulation activation leading to thrombotic events. Tang et al 1 discussed the importance of high D‐dimer and fibrin degradation product level to determine the patient prognostic and the risk of thrombosis. However, they did not look at lupus anticoagulant (LAC). Zhang et al described three cases of thrombosis associated with antiphospholipid antibodies represented by anticardiolipin (aCL) and anti–β2‐glycoprotein I (aβ2GPI). 2 No lupus anticoagulant was detected in any of the patients. During the recent Covid‐19 outbreak in Mulhouse, France, we have studied 56 patients diagnosed for Covid‐19 using polymerase chain reaction (n = 50) or chest computed tomography scan (n = 6), for the presence of LAC with dilute Russell’s viper venom time and sensitive activated partial thromboplastin time tests. Twenty‐five cases (45%) were LAC positive, whereas aCL or aβ2GPI were detected in only five of 50 tested patients (10%, three associated to LAC) using immunoglobulin G and immunoglobulin M detection. Acute infections are known to be sometimes associated with transient LAC, and anticoagulant therapy is usually not needed. 3 Detection of LAC with or without aCL or aβ2GPI, in these critically patients, which are characterized by having many thrombosis risk factors, highlight the importance of an early anticoagulant therapy. CONFLICT OF INTEREST The authors declare that they have no conflicts of interest. AUTHOR CONTRIBUTIONS Inès Harzallah and Bernard Drénou collected the data and processed statistics. Inès Harzallah wrote the manuscript and Bernard Drénou and Agathe Debliquis revised the manuscript.
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              Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients

              R Cervera, R. Serrano, G J Pons-Estel … (2015)
              Objectives To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later. Methods In 1999, we started an observational study of 1000 APS patients from 13 European countries. All had medical histories documented when entered into the study and were followed prospectively during the ensuing 10 years. Results 53.1% of the patients had primary APS, 36.2% had APS associated with systemic lupus erythematosus and 10.7% APS associated with other diseases. Thrombotic events appeared in 166 (16.6%) patients during the first 5-year period and in 115 (14.4%) during the second 5-year period. The most common events were strokes, transient ischaemic attacks, deep vein thromboses and pulmonary embolism. 127 (15.5%) women became pregnant (188 pregnancies) and 72.9% of pregnancies succeeded in having one or more live births. The most common obstetric complication was early pregnancy loss (16.5% of the pregnancies). Intrauterine growth restriction (26.3% of the total live births) and prematurity (48.2%) were the most frequent fetal morbidities. 93 (9.3%) patients died and the most frequent causes of death were severe thrombosis (36.5%) and infections (26.9%). Nine (0.9%) cases of catastrophic APS occurred and 5 (55.6%) of them died. The survival probability at 10 years was 90.7%. Conclusions Patients with APS still develop significant morbidity and mortality despite current treatment. It is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.
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                Author and article information

                Contributors
                Jesse Y. Dabit: dabit.jesse@mayo.edu
                Maria O. Valenzuela-Almada: ValenzuelaAlmada.Maria@mayo.edu
                Sebastian Vallejo-Ramos: vallejo.sebastian1@mayo.edu
                Alí Duarte-García: duarte.ali@mayo.edu
                Journal
                Journal ID (nlm-ta): Curr Rheumatol Rep
                Journal ID (iso-abbrev): Curr Rheumatol Rep
                Title: Current Rheumatology Reports
                Publisher: Springer US (New York )
                ISSN (Print): 1523-3774
                ISSN (Electronic): 1534-6307
                Publication date (Electronic): 5 January 2022
                Publication date (Print): 2021
                Volume: 23
                Issue: 12
                Electronic Location Identifier: 85
                Affiliations
                [1 ]GRID grid.66875.3a, ISNI 0000 0004 0459 167X, Division of Rheumatology, , Mayo Clinic, ; Rochester, MN USA
                [2 ]GRID grid.66875.3a, ISNI 0000 0004 0459 167X, Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, , Mayo Clinic, ; 200 First Street SW, Rochester, MN 55905 USA
                Article
                Publisher ID: 1038
                DOI: 10.1007/s11926-021-01038-2
                PMC ID: 8727975
                PubMed ID: 34985614
                SO-VID: b0a78250-3769-4e5d-8491-7e037584885b
                Copyright © © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022
                License:

                This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

                History
                Date accepted : 10 June 2021
                Categories
                Subject: Antiphospholipid Syndrome (S Zuily, Section Editor)
                Custom metadata
                issue-copyright-statement © Springer Science+Business Media, LLC, part of Springer Nature 2021

                ScienceOpen disciplines: Rheumatology
                Keywords: antiphospholipid syndrome,epidemiology
                Data availability:
                ScienceOpen disciplines: Rheumatology
                Keywords: antiphospholipid syndrome, epidemiology

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