Pediatric antiphospholipid syndrome: Clinical and immunologic features of 121 patients in an international registry

Tadej Avčin, Rolando Cimaz, Earl D. Silverman, Ricard Cervera, Marco Gattorno, Stella Garay, Yackov Berkun, Flavio R. Sztajnbok, Clovis A. Silva, Lucia M. Campos, Claudia Saad-Magalhaes, Donato Rigante, Angelo Ravelli, Alberto Martini, Blaz Rozman, Pier Luigi Meroni

Research output: Contribution to journalArticle

Abstract

OBJECTIVES. The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome. PATIENTS AND Methods. A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday. Results. As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vessel thrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologic manifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-β2n I antibodies in 67%, and lupus anticoagulant in 72%. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations. Conclusions. Clinical and laboratory characterization of patients with pediatric an- tiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups.

Original languageEnglish
JournalPediatrics
Volume122
Issue number5
DOIs
Publication statusPublished - Nov 2008

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Antiphospholipid Syndrome
Registries
Pediatrics
Autoimmune Diseases
Skin Manifestations
Antiphospholipid Antibodies
Venous Thrombosis
Thrombosis
Lupus Coagulation Inhibitor
Anticardiolipin Antibodies
Rheumatology
Neurologic Manifestations
Age of Onset

Keywords

  • Antiphospholipid antibodies
  • Antiphospholipid syndrome
  • Pediatrics

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Pediatric antiphospholipid syndrome : Clinical and immunologic features of 121 patients in an international registry. / Avčin, Tadej; Cimaz, Rolando; Silverman, Earl D.; Cervera, Ricard; Gattorno, Marco; Garay, Stella; Berkun, Yackov; Sztajnbok, Flavio R.; Silva, Clovis A.; Campos, Lucia M.; Saad-Magalhaes, Claudia; Rigante, Donato; Ravelli, Angelo; Martini, Alberto; Rozman, Blaz; Meroni, Pier Luigi.

In: Pediatrics, Vol. 122, No. 5, 11.2008.

Research output: Contribution to journalArticle

Avčin, T, Cimaz, R, Silverman, ED, Cervera, R, Gattorno, M, Garay, S, Berkun, Y, Sztajnbok, FR, Silva, CA, Campos, LM, Saad-Magalhaes, C, Rigante, D, Ravelli, A, Martini, A, Rozman, B & Meroni, PL 2008, 'Pediatric antiphospholipid syndrome: Clinical and immunologic features of 121 patients in an international registry', Pediatrics, vol. 122, no. 5. https://doi.org/10.1542/peds.2008-1209
Avčin, Tadej ; Cimaz, Rolando ; Silverman, Earl D. ; Cervera, Ricard ; Gattorno, Marco ; Garay, Stella ; Berkun, Yackov ; Sztajnbok, Flavio R. ; Silva, Clovis A. ; Campos, Lucia M. ; Saad-Magalhaes, Claudia ; Rigante, Donato ; Ravelli, Angelo ; Martini, Alberto ; Rozman, Blaz ; Meroni, Pier Luigi. / Pediatric antiphospholipid syndrome : Clinical and immunologic features of 121 patients in an international registry. In: Pediatrics. 2008 ; Vol. 122, No. 5.
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abstract = "OBJECTIVES. The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome. PATIENTS AND Methods. A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday. Results. As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5{\%}) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60{\%}), arterial thrombosis in 39 (32{\%}), small-vessel thrombosis in 7 (6{\%}), and mixed arterial and venous thrombosis in 3 (2{\%}). Associated nonthrombotic clinical manifestations included hematologic manifestations (38{\%}), skin disorders (18{\%}), and nonthrombotic neurologic manifestations (16{\%}). Laboratory investigations revealed positive anticardiolipin antibodies in 81{\%} of the patients, anti-β2n I antibodies in 67{\%}, and lupus anticoagulant in 72{\%}. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations. Conclusions. Clinical and laboratory characterization of patients with pediatric an- tiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups.",
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AU - Gattorno, Marco

AU - Garay, Stella

AU - Berkun, Yackov

AU - Sztajnbok, Flavio R.

AU - Silva, Clovis A.

AU - Campos, Lucia M.

AU - Saad-Magalhaes, Claudia

AU - Rigante, Donato

AU - Ravelli, Angelo

AU - Martini, Alberto

AU - Rozman, Blaz

AU - Meroni, Pier Luigi

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N2 - OBJECTIVES. The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome. PATIENTS AND Methods. A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday. Results. As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vessel thrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologic manifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-β2n I antibodies in 67%, and lupus anticoagulant in 72%. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations. Conclusions. Clinical and laboratory characterization of patients with pediatric an- tiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups.

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