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Ann Rheum Dis. 2015 Jun;74(6):1011-8. doi: 10.1136/annrheumdis-2013-204838. Epub 2014 Jan 24.

Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients.

Author information

1
Departament of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain.
2
Lupus Unit, Rayne Institute, St Thomas' Hospital, London, UK.
3
Zabludowicz Centre for Autoimmune Diseases, Chaim-Sheba Medical Centre, Sackler Faculty of Medicine Tel Aviv University, Tel-Hashomer, Israel.
4
Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Hospital Carlos Haya, Málaga, Spain.
5
Departament of Infectious Diseases and Rheumatology, Copenhagen University Hospital at Rigshospitalet, Copenhagen, Denmark.
6
3rd Departament of Medicine, University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary.
7
Servizio di Inmunologia Clinica e Allergologia, Spedali Civili, Azienda Ospidaliera, Brescia, Italy.
8
Transfusion and Haemophilia Centre, Hippocration Hospital, Athens, Greece.
9
Unità Operativa Complessa di Reumatologia, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
10
Allergy and Clinical Immunology Unit, Dipartimento di Medicina Interna, IRCCS Istituto Auxologico, Università di Milano, Milan, Italy.
11
Department of Rheumatology and Clinical Immunology, University Medical Centre, Utrecht, The Netherlands.
12
Department of Haematology, Laboratory of Thrombosis and Haemostasis, University Medical Centre, Utrecht, The Netherlands.
13
Laboratory of Clinical Immunology, Clinical Centre of Allergology, Medical University, Sofia, Bulgaria.
14
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
15
Service de Rhumatologie, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
16
Laboratoire dHématologie, CHU, Nîmes, France.
17
Service de Médecine Interne, Hôpital Claude Huriez, Université Lille Nord-de-France, Lille, France.
18
Unidade de Imunologia Clínica, Hospital Santo António, Centro Hospitalar do Porto and UMIB, ICBAS, Universidade do Porto, Portugal.
19
Department of Rheumatology, Hospital Regional Universitario de Málaga, University of Málaga, IBIMA, Málaga, Spain.
20
Department of Internal Medicine, French National Reference Center for Lupus and Antiphospholipid Syndrome, Hôpital Pitié-Salpetrière, Paris, France.
21
First Department of Internal Medicine, University of Athens School of Medicine, Athens, Greece.

Abstract

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.

KEYWORDS:

Anticardiolipin Antibodies; Antiphospholipid Antibodies; Antiphospholipid Syndrome; Autoimmune Diseases; Systemic Lupus Erythematosus

PMID:
24464962
DOI:
10.1136/annrheumdis-2013-204838
[Indexed for MEDLINE]
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