Published online October 27, 2008
PEDIATRICS Vol. 122 No. 5 November 2008, pp. e1100-e1107 (doi:10.1542/peds.2008-1209)

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ARTICLE

Pediatric Antiphospholipid Syndrome: Clinical and Immunologic Features of 121 Patients in an International Registry

Tadej Avin, MD, PhD^a, Rolando Cimaz, MD^b, Earl D. Silverman, MD, FRCPC^c, Ricard Cervera, MD, PhD, FRCP^d, Marco Gattorno, MD^e,f, Stella Garay, MD^g, Yackov Berkun, MD^h,i, Flavio R. Sztajnbok, MD^j,k, Clovis A. Silva, MD, PhD^l, Lucia M. Campos, MD, PhD^l, Claudia Saad-Magalhaes, MD, PhD^m, Donato Rigante, MDⁿ, Angelo Ravelli, MD^e,f, Alberto Martini, MD^e,f, Bla Rozman, MD, PhD^o and Pier Luigi Meroni, MD, PhD^p,q

^a Department of Allergology, Rheumatology, and Clinical Immunology, University Children's Hospital
^o Department of Rheumatology, University Medical Center, Ljubljana, Slovenia
^b Department of Pediatrics, Meyer Hospital, University of Florence, Florence, Italy
^c Division of Rheumatology, SickKids Hospital, University of Toronto, Toronto, Ontario, Canada
^d Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Catalonia, Spain
^e Pediatria II, Istituto G. Gaslini, Genoa, Italy
^f Department of Pediatrics, University of Genoa, Genoa, Italy
^g Rheumatology Unit, Hospital Sor Maria Ludovica, La Plata, Buenos Aires, Argentina
^h Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Israel
ⁱ Department of Pediatrics, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
^j Rheumatology Section, Adolescent Health Care Unit, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil
^k Department of Pediatric Rheumatology, Universade de Estado do Rio de Janeiro, Rio de Janeiro, Brazil, Unigranrio, Brazil
^l Department of Pediatrics, Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
^m Department of Pediatrics, Botucatu Medical School, Sao Paulo State University, Botucatu, Brazil
ⁿ Department of Pediatric Sciences, Universita’ Cattolica Sacro Cuore, Rome, Italy
^p Department of Internal Medicine, University of Milan, Milan, Italy
^q Allergy, Clinical Immunology, and Rheumatology Unit, San Luca Hospital, IRCCS Istituto Auxologico Italiano, Milan, Italy

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-β₂-glycoprotein 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 antiphospholipid 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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Key Words: antiphospholipid syndrome • antiphospholipid antibodies • pediatrics

Abbreviations: aPL—antiphospholipid antibody • APS—antiphospholipid syndrome • Ped-APS Registry—European registry (extended internationally) of pediatric patients with APS • aCL—anticardiolipin antibody • β₂GPI—β₂ glycoprotein I antibody • LA—lupus anticoagulant • ANA—antinuclear antibody • ENA—extractable nuclear antigen • dsDNA—double-stranded DNA • DVT—deep vein thrombosis • SLE—systemic lupus erythematosus

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Accepted Jul 21, 2008.

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