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; L Ceberio-Hualde; Y Shoenfeld; E de Ramón; V Buonaiuto; Søren Jacobsen; M M Zeher; T Tarr; A Tincani; M Taglietti; G Theodossiades; E Nomikou; M Galeazzi; F Bellisai; P L Meroni; R H W M Derksen; P G D de Groot; M Baleva; M Mosca; S Bombardieri; F Houssiau; J-C Gris; I Quéré; E Hachulla; C Vasconcelos; A Fernández-Nebro; M Haro; Z Amoura; M Miyara; M Tektonidou; G Espinosa; M L Bertolaccini; M A Khamashta; Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)

doi:10.1136/annrheumdis-2013-204838

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, L Ceberio-Hualde, Y Shoenfeld, E de Ramón, V Buonaiuto, Søren Jacobsen, M M Zeher, T Tarr, A Tincani, M Taglietti, G Theodossiades, E Nomikou, M Galeazzi, F Bellisai, P L Meroni, R H W M Derksen, P G D de Groot, M BalevaM Mosca, S Bombardieri, F Houssiau, J-C Gris, I Quéré, E Hachulla, C Vasconcelos, A Fernández-Nebro, M Haro, Z Amoura, M Miyara, M Tektonidou, G Espinosa, M L Bertolaccini, M A Khamashta, Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)

Department of Clinical Medicine

301 Citations (Scopus)

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.

Original language	English
Journal	Annals of the Rheumatic Diseases
Volume	74
Issue number	6
Pages (from-to)	1011-1018
Number of pages	8
ISSN	0003-4967
DOIs	https://doi.org/10.1136/annrheumdis-2013-204838
Publication status	Published - 1 Jun 2015

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Cervera, R., Serrano, R., Pons-Estel, G. J., Ceberio-Hualde, L., Shoenfeld, Y., de Ramón, E., Buonaiuto, V., Jacobsen, S., Zeher, M. M., Tarr, T., Tincani, A., Taglietti, M., Theodossiades, G., Nomikou, E., Galeazzi, M., Bellisai, F., Meroni, P. L., Derksen, R. H. W. M., de Groot, P. G. D., ... Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies) (2015). Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Annals of the Rheumatic Diseases, 74(6), 1011-1018. https://doi.org/10.1136/annrheumdis-2013-204838

Cervera, R, Serrano, R, Pons-Estel, GJ, Ceberio-Hualde, L, Shoenfeld, Y, de Ramón, E, Buonaiuto, V, Jacobsen, S, Zeher, MM, Tarr, T, Tincani, A, Taglietti, M, Theodossiades, G, Nomikou, E, Galeazzi, M, Bellisai, F, Meroni, PL, Derksen, RHWM, de Groot, PGD, Baleva, M, Mosca, M, Bombardieri, S, Houssiau, F, Gris, J-C, Quéré, I, Hachulla, E, Vasconcelos, C, Fernández-Nebro, A, Haro, M, Amoura, Z, Miyara, M, Tektonidou, M, Espinosa, G, Bertolaccini, ML, Khamashta, MA & Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies) 2015, 'Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients', Annals of the Rheumatic Diseases, vol. 74, no. 6, pp. 1011-1018. https://doi.org/10.1136/annrheumdis-2013-204838

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

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.",

author = "R Cervera and R Serrano and Pons-Estel, {G J} and L Ceberio-Hualde and Y Shoenfeld and {de Ram{\'o}n}, E and V Buonaiuto and S{\o}ren Jacobsen and Zeher, {M M} and T Tarr and A Tincani and M Taglietti and G Theodossiades and E Nomikou and M Galeazzi and F Bellisai and Meroni, {P L} and Derksen, {R H W M} and {de Groot}, {P G D} and M Baleva and M Mosca and S Bombardieri and F Houssiau and J-C Gris and I Qu{\'e}r{\'e} and E Hachulla and C Vasconcelos and A Fern{\'a}ndez-Nebro and M Haro and Z Amoura and M Miyara and M Tektonidou and G Espinosa and Bertolaccini, {M L} and Khamashta, {M A} and {Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)}",

note = "Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.",

year = "2015",

month = jun,

day = "1",

doi = "10.1136/annrheumdis-2013-204838",

language = "English",

volume = "74",

pages = "1011--1018",

journal = "Annals of the Rheumatic Diseases",

issn = "0003-4967",

publisher = "B M J Group",

number = "6",

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TY - JOUR

T1 - Morbidity and mortality in the antiphospholipid syndrome during a 10-year period

T2 - a multicentre prospective study of 1000 patients

AU - Cervera, R

AU - Serrano, R

AU - Pons-Estel, G J

AU - Ceberio-Hualde, L

AU - Shoenfeld, Y

AU - de Ramón, E

AU - Buonaiuto, V

AU - Jacobsen, Søren

AU - Zeher, M M

AU - Tarr, T

AU - Tincani, A

AU - Taglietti, M

AU - Theodossiades, G

AU - Nomikou, E

AU - Galeazzi, M

AU - Bellisai, F

AU - Meroni, P L

AU - Derksen, R H W M

AU - de Groot, P G D

AU - Baleva, M

AU - Mosca, M

AU - Bombardieri, S

AU - Houssiau, F

AU - Gris, J-C

AU - Quéré, I

AU - Hachulla, E

AU - Vasconcelos, C

AU - Fernández-Nebro, A

AU - Haro, M

AU - Amoura, Z

AU - Miyara, M

AU - Tektonidou, M

AU - Espinosa, G

AU - Bertolaccini, M L

AU - Khamashta, M A

AU - Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)

N1 - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

PY - 2015/6/1

Y1 - 2015/6/1

N2 - 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.

AB - 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.

U2 - 10.1136/annrheumdis-2013-204838

DO - 10.1136/annrheumdis-2013-204838

M3 - Journal article

C2 - 24464962

SN - 0003-4967

VL - 74

SP - 1011

EP - 1018

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

IS - 6

ER -

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

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