Pediatric Rheumatology Online Journal → July 2003 → Anti-Phospholipid Antibody Syndrome→ Abstract #116

LONG TERM TREATMENT OF ANTI PHOSPHOLIPID SYNDROME (APS) ASSOCIATED CEREBRAL ARTERIAL THROMBOSES WITH INTRAVENOUS IMMUNOGLOBULIN (IV IG)

B. Arabshahi,¹ E. D. Thompson,¹ E. M. Smergel,¹ D. P. Goldsmith.¹

Section of Rheumatology, St. Christopher's Hosp. for Children/Drexel University College of Medicine, Philadelphia, PA; Section of Rheumatology, St. Christopher's Hosp. for Child/Drexel University College of Medicine, Philadelphia, PA; Section of Radiology, St. Christopher's Hosp for Child/Drexel University of Medicine, Philadelphia, PA; Section of Rheumatology, St. Christopher's Hosp for Child/Drexel University College of Medicine, Philadelphia, PA

There is both laboratory and clinical evidence for the benefical role of IVIg in APS. Most previous reports however, focus on the use of IVIg for obstetric complications. The presence of antiphospholipid antibodies (APL) is a significant risk factor for ischemic cerebral arterial disease in children with the current therapeutic recommendation being long-term anticoagulation with warfarin. In certain clinical situations however, this therapy may invoke too high a risk and necessitate the need for alternative therapeutic interventions.
We report a 5 year-old male with Trisomy 21, hypothyroidism, and insulin dependent diabetes, who developed acute hemiplegia. There were multiple cerebral infarcts with MRA evidence of right anterior and middle cerebral artery occlusion and a Moya-Moya pattern (hemiplegia with supraclinoid carotid stenosis and multiple cerebral telangiectasia). Protein S,C, and anti-thrombin III levels were normal, and factor V Leiden assay was negative. The IgG anticardiolipin antibody (ACL) level was markedly elevated to 116 GPL units; IgM and IgA APL were negative. Significant behavioral issues and problems with balance coupled with the Moya-Moya arterial pattern precluded maintenance treatment with warfarin. A monthly IVIg infusion (2g/kg) was started and within 4 months the IgG ACL level fell to 28 GPL units, becoming normal 6 months later. IVIg was administered at 8 week intervals over the next 7 years with continued normal IgG ACL levels and no clinical CNS deterioration. Serial MRA studies showed no progression of cerebral arterial occlusive disease.
We suggest that IVIG may be an alternative therapeutic choice to prevent further arterial thrombotic events in those children with APS who are not candidates for conventional anticoagulation.