High-risk patients Low-risk patients Lupus anticoagulant presence Isolated intermittently positive aCL or aβ2GPI at low titers aCL at medium–high titers Previous single venous thrombosis Double or triple positivity (LA, aCL,…
Fig. 13.1 The potential involvement of the eye, ear-nose-throat, lung, gastrointestinal system, and endocrine glands in APS is illustrated 13.2 Eye Involvement in APS While the eye involvement is uncommon…
Treatment Response rate Sustained response First-line therapy Corticosteroids ~70–85 % 20–30 % Prednisone 1 mg/kg/day p.o. Methylprednisolone i.v. 5–10 mg/kg/day for 3–5 days High-dose dexamethasone 40 mg/day for 4…
Acute lesions Chronic lesions Thrombotic microangiopathy (TMA) Arterial fibrous intimal hyperplasia (FIH) or arterial occlusion by organized thrombi with or without recanalization Focal cortical atrophy (FCA) Tubular thyroidization APS nephropathy…
Clinical manifestation Association Level of evidence Dementia Association between LA and dementia Level II Cognitive dysfunction Association between aPL and cognitive dysfunction Level I Migraine No association between migraine and…
Criteria for obstetric APS 1. One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with normal fetal morphology documented by ultrasound…
© Springer International Publishing Switzerland 2015Pier Luigi Meroni (ed.)Antiphospholipid Antibody SyndromeRare Diseases of the Immune System10.1007/978-3-319-11044-8_1 1. Genetics and Origin of Antiphospholipid Syndrome Alessandra Soriano1, 2 , Miri Blank3 and Yehuda Shoenfeld1, 4 (1) Zabludowicz Center for Autoimmune…
Test Distinctive features Anti-PS/PT antibodies Strong correlation with LA Association with obstetric or thrombotic manifestations not definitely demonstrated Anti-DI antibodies Association with triple positivity Association with thrombosis (in few studies)…
Clinical Serology 1. Venous thrombosis 1. IgG anticardiolipin antibody (>20 GPL units) 2. Arterial thrombosis 2. Positive test for lupus anticoagulant 3. Recurrent fetal loss 3. IgM anticardiolipin antibody (>20…
© Springer International Publishing Switzerland 2015Pier Luigi Meroni (ed.)Antiphospholipid Antibody SyndromeRare Diseases of the Immune System10.1007/978-3-319-11044-8_4 4. The Pathogenic Mechanisms for Antiphospholipid Antibodies (aPL)-Mediated Pregnancy Loss Nicoletta Di Simone1 and Silvia D’Ippolito1 (1) Department of Obstetrics and…
