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 language | English |
|---|---|
| Journal | Pediatrics |
| Volume | 122 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Nov 2008 |
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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 journal › Article
}
TY - JOUR
T1 - Pediatric antiphospholipid syndrome
T2 - Clinical and immunologic features of 121 patients in an international registry
AU - Avčin, Tadej
AU - Cimaz, Rolando
AU - Silverman, Earl D.
AU - Cervera, Ricard
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
PY - 2008/11
Y1 - 2008/11
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.
AB - 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.
KW - Antiphospholipid antibodies
KW - Antiphospholipid syndrome
KW - Pediatrics
UR - http://www.scopus.com/inward/record.url?scp=58149149373&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=58149149373&partnerID=8YFLogxK
U2 - 10.1542/peds.2008-1209
DO - 10.1542/peds.2008-1209
M3 - Article
C2 - 18955411
AN - SCOPUS:58149149373
VL - 122
JO - Pediatrics
JF - Pediatrics
SN - 0031-4005
IS - 5
ER -