Index

Abortions, 4, 120-12l, see also Pregnancy loss

Abramowsky, C. R., 283 Abu Shakra, M., l3 aCL, see Anticardiolipin

antibodies aCLlaPS FACS Kit, 210 Acrocyanosis, 67 Actin FS, 231 Activated partial thromboplastin

time (aPTT), 231, 247 bone marrow transplantation

and,382 CPZ-induced aPL and, 145 DRVVTand,219 KCT and, 217-18 LA measurement and, 215-17,

222 modified,217 MRL/lpr models and, 381 pregnancy and, 187 procainamide-induced aPL

and,148 Acute rheumatic fever (ARF),

156-7 Addison's disease,S, 450

APS in children and, 160 Adenosine diphosphate (ADP),

291,293 drug-induced aPL and, 151 thrombocytopenia and, 411-12

Adult respiratory distress syndome (ARDS), l30-2

AIDS aCL testing and, 205-6 secondary APS and, 15 thrombocytopenia and, 413

AIHA (autoimmune hemolytic anemia), 15,23-4,272

Aird, W. c., 450 Alarcon-Segovia, D., 6, 99, 116 Albengres, E., 433 Alleles, 351-6

polymorphism and, 362-3 thrombocytopenia and, 412

Alzheimer's disease, 105 Ames, P. R. J., 7l, 249, 449-56 Amengual, 0., 245-52 American College of Chest

Physicians, 187 American College of Rheumatology

(ACR), 3, 264

American Physicians Health Study, 12

Amigo, M. C., 70-81 Amital, H., 34 Amoxycillin, 149 ANA, see Antinuclear antibody Ancrod, l31 Andrews, P. A., 113 Anemia, 9 Angelini, L., 160-1 Angina, 36 Angiography, 44 Angioplasty, 38 Angles-Cano, E., 325-36 Annexin, 211

endothelial cells and, 304 fibrinolysis and, 329 LAand,226 placental pathology and,

286-7 tissue factor and, 320-1

Antibodies, 21, 99, 459, 462 AECA, 300, 304 annexin V, 211 antiprothrombin, 245-52 antithrombomodulin, 311-12 APC resistance and, 312-14 aPL assays and, 254 auto, 254, 256-8 BFP-STS and, l36 bivalent antigen complexes

and,232 bone marrow transplantation

and,382 catastrophic APS and, l32 drug-induced aPL and, 150-2 fibrinolysis and, 326-7 fiourescent, 263 glycoprotein measurement and,

238-44 immunoassays and, 254-56 immunomodulation and, 384 infertility and, 188-9, 432 IVF and, 430 MOAB,228 monoclonal vs. glycoprotein,

230-2 MRLllpr models and, 380-1 PE and, 272-3 renal pathology and, 274 sequence origin and, 360-1 specificity and, 1l3-16

463

thrombocytopenia and, 409-10, 412

see also Antinuclear antibodies (ANA); Antiphospholipid antibodies (aPL)

Anticardiolipin antibodies (aCL), 5,8,11, III

AIHA and, 23-4 angioplasty and, 38 aPL and, l38, 144-6, 149-51,

228,309-10 APS and, l3, 156, 158, 162-3,

264 aPT and, 245, 247, 249 blood donors and, 14 bone marrow transplantation

and,382 cerebral disease and, 43, 46 clinical features and, 16 cognitive dysfunction and, 49 contraceptives and, 438 coronary artery disease and,

36-8 DVTand,12 endothelial cells and, 300 ESRD and, 77 gammaglobulin and, 384 genetics and, 149 glycoprotein and, 238-44 hearing loss and, 52-3 heart and, l3 histocompatibility complex

and, 350, 353 infertility and, 188-90,427 ITP and, 22 LA and, 225-6, 229, 231, 233-4 measurement and, 10 migraines and, 51 MRL/lpr models and, 379-82 neuroimaging and, 53-4 pregnancy and, 183-6,282,398 pseudo thrombocytopenia and,

23 psychosis and, 50 pulmonary hypertension and,

98-9 renal transplantation and, 78 secondary APS and, 14-15 seizures and, 51 SLE and, 15 SNHL and, 82, 85-6

464

Anticardiolipin antibodies (aCL) - cont.

solid phase immunoassays for, 136-7

steroids and, 401 testing and, 205-13 thrombocytopenia and, 201,

408-9,412-3 thrombosis and, 13-14, 392 thromboxane and, 341-2 tissue factor and, 319-20 TTP and,26 valvular abnormalities and, 34,

36 Anticardiolipin syndrome, see

Antiphospholipid syndrome (APS)

Anticardiolipin Wet Workshop, 210

Anticoagulation catastrophic APS and, 127-8,

131,133 steroids and, 4 thrombosis and, 9-10 see also Lupus anticoagulant

(LA) Anti-dsDNA, 15 Anti-Ku antibodies, 99 Antimitochondrial antibodies, 21 Antinuclear antibodies (ANA),

113 catastrophic APS and, 130 cognitive dysfunction and, 49 CPZ-induced aPL and, 146 genetics and, 149 infertility and, 190,426-7 PAPS and, 15 procainamide and, 144

Antiphosphatidyinositol, 189 Antiphosphatidylserine, 189 Antiphospholipid antibodies

(aPL), 5, 8, 17 AIHA and, 23-4 anti-platelet antibodies and,

21-2 APC resistance and, 312-3 apoptosis and, 372-8 atherosclerosis and, 167-9,

172-8 cardiomyopathy and, 39 catastrophic APS and, 127-34 cerebral disease and, 43 cerebral ischemia and, 44-6 children and, 155-66 chorea and, 52 cognitive dysfunction and,

47-50 coronary artery disease and,

36-8 cortical renal ischemia and,

76-7 DICand,26 drug-induced,144-54 eicosanoids and, 337-47 endothelial cells and, 299-306 ESRD and, 77 fibrinolysis and, 330-2

flow cytometry and, 210 Guillain-Barre Syndrome and,

52 HIVand,9 infections and, 135-43 infertility and, 427-30 rTP and, 22-3 rVF and, 431 kidney disease and, 70-81 LA and, 215, 225-6, 233-4 measurement and, 10 migraines and, 51 monoclonal, 358-71 MRL/lpr models and, 380 neuroimaging and, 53-4 other hemocytopenias and,

26-7 other phospholipids and,

253-60 PAPS and, 111-26 pathogenic mechanism of,

226-28 placental pathology and, 286-7 pregnancy and, 282 pregnancy loss and, 179-94 prevalance of, 11-12 protein C and, 307 psychosis and, 50 pulmonary hypertension and,

98-101 risk of positive tests and, 12-13 seizures and, 51 skin manifestations and, 59 Sneddon's syndrome and, 48 specificity of, 309-10 spinal cord and, 106 thrombocytopenia and, 20-2,

410-12 thrombosis and, 13-14, 290,

292,294-5, 392 transverse myelopathy and, 52 treatment and, 54-5 TTP and, 25-6 valvular disease and, 32-6 venous sinus thrombosis and,

47 Antiphospholipid Antibodies in

Stroke Study (APASS) Group, 11-12,36, 119

Antiphospholipid syndrome (APS)

aCL and, 13, 136, 205-13, see also Anticardiolipin antibodies

aPL prevalence and, 11-13, see also Antiphospholipid antibodies

aPT and, 245-52 atherosclerosis and, 167-78 cardiac manifestations and,

32-42 catastrophic, 121, 127-34,

451-2 cerebral disease and, 43-58 children and, 155-66 classification and, 8-9 clinical featuers of, 16-17

critical illness and, 419-26 demographics and, 8 differential diagnosis and,

449-56 ear and, 82-8

Index

eicosanoids and, 337-47 endothelial cells and, 299-306 experimental models and,

379-88 eye and, 89-95 fibrinolysis and, 325-36 hemocytopenias and, 20-31 histocompatibility complex

and,348-57 hormone replacement and,

439-41 infectious aPL and, 135-43 infertility and, 426-32 kidney disease and, 70-81 LA and, 214-37, see also Lupus

anticoagulant measurement and, 9-10 micro emboli imaging and,

195-201 monoclonal aPL and, 358-71 oral contraceptives and, 432-41 other phospholipids and,

253-60 placental pathology and, 281-9 plasminogen activation and,

325-6 pregnancy and, 397-407 pregnancylossand,179-94 primary, 8,15,39,49,71-5,

77-9,85,111-26 prognosis and, 459-62 protein C pathway and, 307-16 pulmonary hypertension and,

96-104 secondary, 14-15,48-9,86,355 skin manifestations and, 59-69 thrombocytopenia and, 408-18 thrombosis and, 13-14, 290-8,

391-6 tissue-factor pathway and,

317-24 vascular pathology of, 263-80

Anti-platelet antibodies, 21-2, 55 Antiprothrombin (aPT), 245-6,

251 clinical significance, 248-9 history of, 247 immunological characteristics,

247-8 pathogenic role of, 249-50

Antithrombin III deficiency, 14 Aoki, K., 188-9,256 Aortic valve, 33 APACHE scores, 420, 423 APC, 308-14 APhL ELISA kit, 208-9, 211 aPL, see Antiphospholipid

antibodies Apolipoprotein E, 167 Apoptosis, 359, 377

aPL and, 375-6 atherosclerosis and, 167

Index

description of, 372-3 dying cells and, 373-5 occurrence of, 373

APS, see Antiphospholipid syndrome

aPTT, see Activated partial thromboplastin time

Arachidonic acid, 226, 337, 339 Arachnase,222,231 ARDS, 130-132 Arg, 246-7, 320

proteins and, 309 thrombosis and, 293

Arginyl, 327 Arnett, F. c., 354 Arnout, j., 225-37, 295, 342 Arterial disease, 13-14 Arterial thrombosis, 3-4, 11,

450-1 Arvieux, j., 342, 411 Asherson, R. A., 5, 15,37,52,71,

89,94,99, 113, 127-8,355 Aspirin, 55, 110, 393

IVF and, 431-2 pregnancy and, 187,399-400

Ataxia, 82 Atheroma, 172 Atherosclerosis, 36-8

accelerated, 172-8 aPL and, 168-9 etiologic factors and, 167-8 lupus and, 168

Atsumi, T., 245-52, 313 Autoimmune disorders

apoptosis and, 373-6 APS and, 3, 156 BFP-STS and, 136 CPZ-induced aPL and, 146 critical illness and, 420, 422 drug-induced aPL and, 149 histocompatibility complex

and,348-57 infectious aPL and, 137-8 IVF and, 430-2 MRLllpr models and, 381 myocardial infarction and, 38 other phosopholipids and,

254-6 procainamide-induced aPL

and, 147-8 SNHL and, 82-4

Autoimmune hemolytic anemia (AIHA), 15,23-4,272

Avascular necrosis, 16 Azathioprine, 401

Baca, V., 163 Balasch, j., 430 Barbui, T., 408-18 Battagliotti, C. A., 59-69 Bauersachs, R., 437-8 Beaumont, j. L., 6 Beaumont, V., 433, 436 Becker, R. c., 36 Behcet's disease, 5, 450 Belmont, H. M., 127-34

Ben-David, j., 82-8 Bentolia, S., 355 Bertolaccini, M. L., 245-52 Berube, C., 158 Bevers, E. M., 247 Bewley, S., 397-407 BFP-STS, 5-6, 136

aCL testing and, 206 histocompatibility complex

and,349-50,354 LA and, 225

Bias, W. B., 350 Biospecific interaction analysis

(BIA),228 Birdsall, M. A., 430 Birkenfeld, A., 427, 431 Bivalent antigen-antibody

complexes, 232 Blank, M., 342, 379-88 Bloemenkamp, K. W. M., 433-4,

437 Blood,108

aCL and, 14 antibodies and, 114-15 livedo reticularis and, 60 pressure, 4 see also Platelets

Boffa, Marie Claire, 6 Bone marrow transplantation,

382-3,385 Bongard, 0., 12 Bos, K., 127 Boston Collaborative Drug

Surveillance Program, 439 Bowel infarction, 5 Bowie, E. j. W., 6, 136,225 Brain

disorders of, 105-7 endothelial cells and, 302

Branch, D. W., 179-194,281,380, 430

Brey, R. L., 43-58 Brezinschek, H. P., 362 British Society for Haematology,

221 Bromocriptine (BRC), 385 Brown, j. H., 39 Brown Recluse spider, 222 Brunet, P., 77 Budd-Chiara syndrome, 4-5

APS in children and, 160 catastrophic APS and, 128

Buyon, j., 441 Byron, Margaret, 5

C4Bp,313 C4 levels, 15 C5-9 formation, 295 C5b-9, 291 Cabiedes, j., 115 Cacoub, P., 76 CADASIL,451 CAD (coronary heart disease),

168,198 Calcification, 33 Calcinosis, 98 Calciphylaxis, 269, 271

Calcium, 162, 187 Caldarelli, D. D., 83 Campbell, A. L., 137 Camps, M. T., 353 Capillaritis,272 Caporali, R., 20-31 Captropril, 128 Carbosylation, 246

465

Carbotte, R. M., 49 Carboxyfluorescein, 137 Cardiolipin, 23, 135-6,341,358

see also Anticardiolipin antibodies

Cardiomyopathy, 16, 39 Cardiovascular system, 119 Carreras, L. 0.,226,299,337-47 Casciola-Rosen, L., 359 Casoli, P., 86 Castanon, c., 89-95 CD62,291 Celli, M., 137 Cell lysis, 167 Cerebral disease, 43

dementia, 47-50 ischemia, 4, 9, 44-6, 48,

197-200 migraine, 50-1 psychosis, 50 seizures, 51 treatment and, 54 venous thrombosis, 47

Cerebral vascular accidents (CVA), 149,302

Cerebrospinal fluid (CSF), 451 Cerebrovascular ischemia (CVI),

197-200 Cerebral venous sinus thrombosis

(CVT),47 Cervera, R., 32, 51 Cesbron, j -Y, 49 Chakravarty, K. K., 12 Chan, john, 5 Chemoattraction, 167 Chemokines, 167 Children

APS treatment and, 162-3 clinical associations of aPL and,

155-7 neonatal effects of aPL and,

161-2 PAPS and, 162 SLE and, 157-9 thrombosis and, 159-61

Chlorpromazine drug-induced aPL and, 145-7,

149,150-1 genetics and, 149-50 infectious aPL and, 137

Cholangiopancreatography, 128 Cholesterol, 13 Chorea, 4-5, 51-2

aCL testing and, 211 APS in children and, 159 CNS and, 120 contraceptives and, 438 treatment and, 54

Ciprofloxacin, 385

466

CNS (central nervous system) APS and, 130, 158, 195 cerebral disease and, 43 endothelial cells and, 301 neuroimaging and, 53 PAPS and, 119-20 Sneddon's syndrome and, 48

Coagulation catastrophic APS and, 127-28,

131,133 myocardial infarction and, 37 thrombosis and, 392-5 see also Lupus anticoagulant

Cogan's syndrome, 82, 86 Cognitive dysfunction, 47

aPL associated, 48-9 medication-induced, 49-50 Sneddon's syndrom and, 48

Colaco, Bernie,S College of American Pathologists,

207 Colloidal silica (CSCT), 218 Coma, 43 Conley, C. L., 155, 205 Contraceptives

APS and, 438-41 arterial thromboembolism and,

435-7 catastrophic APS and, 128 chorea and, 51-2 hormone replacement and,

439-41 hypercoagulability and, 432-3,

437-8 venous thromboembolism and,

433-5 Contractor, S., 162 Coombs' test, 13, 20

AIHA and, 23 APS in children and, 159 lIP and, 22

Coronary artery disease, 36-8 Cortical renal ischemia, 76-7 Corticosteroids

cerebral ischemia and, 46 chorea and, 52 cognitive dysfunction and,

49-50 transverse myelopathy and, 52 valvular disease and, 32

Coulam, C. B., 429-30, 432 Coumadin,110 Cowchock F. S., 401 Cox, ). P. L., 362 CPZ

drug-induced aPL and, 145-7, 150-1

genetics and, 149-50 C-reactive protein, 36 Creatsas, G., 433 CREST, 98 Crohn's disease, 67 Crossed-immunoelectrophoresis

(CIE),247 Cryoglobulinemia, 63 CTD (connective tissue

disorders), 98-9, 101

CT scan catastrophic APS and, 130 cortical renal ischemia and, 76

Cuadrado, M. J., 317-24 Cubillos, )., 429 Currie, A. R., 372 Cutaneous gangrene, 63 Cyclo-oxygenase (COX), 337-8,

343-4 Cytokines, 132 Cytomegalovirus (CMV), 15, 136,

139

DADE-Behring, 231 Dahlback, B., 309 Davis, S., 147 D'Cruz, D., 8-19 Deep vein thromboembolism

(DVT), 11, 149 aCL and, 12 APS in children and, 157-9 APS pathology and, 271 aPT and, 249 catastrophic APS and, 128, 130 CPZ-induced aPL and, 147 critical illness and, 421 procainamide-induced aPL

and, 147 Defibrotide, 131 de Groot, P. G., 307-16 de Kleyn, A., 85 Deleze, M., 24 Del Papa, N., 299-306, 343 Dementia,S, 43, 47

cognitive dysfunction and, 4e-50

Sneddon's syndrome and, 48 treatment and, 54

de Moerloose, P., 49 Denburg, S. D., 48 Denis, A. L., 430 Depression, 50 Derksen, R. H. W. M., 307-16 Defue, Genevieve, 5 Devor, M., 439 Diabetes

APS in children and, 157 Guillain-Barre Syndrome and,

52 migraines and, 51 skin manifestations and, 63 TTP and, 26

Diastolic dysfunction, 16 Diaz, M. N., 36 Dilute prothrombin time (dPT),

231 Dilute Russell's viper venom time

(DRVVT), 215, 231 APS pathology and, 264 LA measurement and, 217-22

Dilute thromboplastin time test (DTT),218

Diphenylhydantoin, 149 Disseminated intravascular

coagulation (DIC), 25-6 aPL and, 26

catastrophic APS and, 131 tissue factor and, 318

DNA

Index

apoptosis and, 375 atherosclerosis and, 169 contraceptives and, 438 drug-induced aPL and, 150 infertility and, 427 monoclonal aPL and, 366, 368 MRLllpr models and, 381

Dobado-Berrios, P. M., 317-24 Donohoe, S., 214-24 Doppler imaging, 32-4,197-200 Dosekun, A. K., 127 Drugs

aPL inducement and, 145-54 pregnancy and, 187-8 SLE inducement and, 144-5

dsDNA, 35, 113 catastrophic APS and, 132 monoclonal aPL and, 358

DVT, see Deep venous thromboembolism

Dyskinesia, 151

Ear, 107 immunological profile and, 84 SNHL and, 82-3, 85-6

Eber, B., 38 Ecarin, 220-1 Echis carinatus, 220 Echocardiography, 32-4

cerebral ischemia and, 44 E. coli, 422 Edson, J. R., 247 EDTA (ethylene diamine tetra­

acetic acid), 23 Eicosanoids

aPL and, 339-45 background of, 339-41 biosynthesis of, 337-9 prostacyc1in and, 343-4 thromboxane and, 341-2

ELISA (enzyme-linked immunoabsorbent assay), 5,84

aCL and, 136-7,205,207-11 aPL specificity and, 309-10 aPT and, 245, 247 atherosclerosis and, 169 autoantibodies and, 254, 256-8 drug-induced aPL and, 146,

150 immunoassays and, 254-6 LA and, 214 protein C and, 307 thromboxane and, 342

Elkon, K. B., 5, 372-8 Embryonic period, 180 Emlen, W., 14 Endarterectomy, 197 Endocardial valve disease, 113 Endocarditis, 33-6 Endocrine system, 109 Endometriosis, 189-90 Endothelial cells, 459-60

aPL and, 151, 227, 299-306, 317

Index

apoptosis and, 303-4 APS and, 132-3,273 aPT and, 249 fibrinolysis and, 325-6, 329-30,

333 prostacyclin and, 343-4 thrombocytopenia and, 411 tissue factor and, 319 TMA and, 263, 269

End-stage renal disease (ESRD), 77,79

Enzyme immunoassays (EIA), 238,264

Epidermal growth factor (EGF), 327

Epilepsy, 4-5 Epiphenomenon, 10 Epitopes, 232, 240

aPT and, 245, 248 endothelial cells and, 303

Epstein-Barr virus aCL and, 137 aPL and, 139 monoclonal aPL and, 359

Erythematosus plaques, 63 Escherichia coli, 422 E-selectin, 302, 343, 384 Esophageal dysmotility, 98 ESRD (end-stage renal disease),

77,79 European Concerted Action of

Thrombosis (ECAT), 231 Evans' syndrome, 24, 108 Exner's test, 217-18 Eye, 107

clinical studies and, 89-90 discussion of, 93-4 ocular findings and, 90-2

Fab' fragments, 228-9, 232 thrombosis and, 293 thromboxane and, 342 tissue factor and, 320

Factor Va, 309 Factor V Leiden, 14

contraceptives and, 433-5, 437 CVT thrombosis and, 47 pregnancy loss and, 452

Factor X activation, 206 Fanelli, A., 121,411 Fanopoulos, D., 241 Fas deficiency, 376-7 Fcg receptor, 341-2 FDA,21O Feichtinger, W., 431 Feinstein, D. 1., 225 Fetal loss, 4-5

antibodies and, 115-16 catastrophic APS and, 129 placental pathology and, 281-9 see also Pregnancy loss

Fibrin MRL/lpr models and, 380 skin manifestations and, 63 TAFI and, 308 transverse myelopathy and, 52

Fibrinogen, 219

Fibrinolysis endothelial cells and, 329-30 new directions for, 332-3 protein interactions and, 327-9 thrombosis and, 392-5 vascular wall and, 325-7

Fibrosis, 73 cortical renal ischemia and, 76 valvular disease and, 34

Fields, R., 11 Fijnheer, R., 312 Finazzi, G., 392 Finger domains, 327-8 Fisher's test, 420 Fishman, P., 385 5H2,232 Fleck, R. A., 247 Flocculation test, 135 Flow cytometry, 210 Fluorangiography, 90 Font, J., 32, 113 Forastiero, R. R., 138 Forbes, C. D., 439 Ford,I.,342 Fulminant encephalopathy, 48

GABA,51 Galli, M., 22, 248, 313, 408-18 Gammaglobulin, 384 Gangrene, 63 Garcia-Torres, R., 34, 70-81 Gattorno, M., 161 Genetics, 8, 10

cerebral ischemia and, 46 drug-induced aPL and, 149-50 human sequences, 365-9 mouse sequences and, 364-5 PAPS and, 15 V sequences and, 362-9

Genitalia, 107 George, J., 38, 343 Geva, E., 430 Gharavi, A. E., 3-6, 90, 135-43,

205-13, 382, 386 Ghirardello, A., 11, 13 Ginsberg, J., 11-12 Giraffe skin, 264 Gleicher, N., 188,427 Glial fibrillary acidic protein

(GFAP),264 Glomerular capillary thrombosis,

71 Glomerulonephritis, 273 Glucocorticoids, 22 Glueck, H. I., 71, 77 Glycoprotein, 5-6, 10,459-60,462

aCL testing and, 207-11 antibodies and, 114-16,238-44 antiprothrombin and, 245-52 APC resistance and, 313 aPL and, 21-2,137-9,150-1,

228, 254, 309-10, 358-9, 365

apoptosis and, 373 atherosclerosis and, 169, 172-5 autoantibodies and, 256-8 cerebral ischemia and, 46

endothelial cells and, 300-4, 343-4

fibrinolysis and, 329-32 gammaglobulin and, 384 histocompatibility complex

and,350

467

immunoassays and, 254-6 infertility and, 189,427,430,432 LA and, 214, 222, 225-6,

228-230,232-4 monoclonal antibodies and,

230-2 MRL/lpr models and, 379-84 myocardial infarction and, 38 other hemocytopenias and, 26 PAPS classification and, 116-17 placental pathology and, 286 pregnancy and, 403 protein C and, 308 pulmonary hypertension and, 99 secondary APS and, 15 thrombocytopenia and, 21,

408-12,454 thrombosis and, 292-5, 391-2 thromboxane and, 341-2 tissue factor and, 319-20

Glycosylphosphatidylinositol, 329 Godeau, B., 22 Godfrey, T., 8-19 Goel, N., 351 Golan, T. D., 82-8 Goldstein, S. R., 180 Gomez-Pacheco, 1., 392 G phospholipid (GPL), 207 Graft occlusion, 44 Granulocyte-monocyte

colony-stimulating factor (GM-CSF),385

Greisman, S. G., 127 Guillain-Barre syndrome, 5, 52,

119 Guy's Hospital, 402 Guzman, J., 24

Hachulla, E., 54 Hage, M. 1., 162 Hammersmith Hospital, 206, 372 Hamsted, A., 36-7 Hanly, J. G., 283 Hanson, S. R., 310-11 Harper, M. F., 229 Harris, E. N., 4-6, 21, 52, 116, 127,

136, 179, 205-13, 264, 269, 372

Harris, P. J., 84 Hartman, R. C., 155,205 Hashimoto's thyroiditis, 136 HDL (high-density lipoproteins),

169 Headaches, 4 Hearing loss, 52-3 Heart, 13, 109, 197 Heart valve disease, 5, 9, 15

clinical manifestations and, 34-6

pathogenesis and, 34 thickening and, 32-4

468

Heinemann, L. A. J., 436 HELLP syndrome, 25

catastrophic APS and, 128, 131 TTP and, 26

Helsinki heart study, 13 Hemocytopenias

autoimmune hemolytic anemia (AIHA) and, 23-4

autoimmune thrombocytopenia and, 20-3

other types of, 26-7 thrombotic and

microangiopathic, 24-6 Hemolytic anemia, 20

APS in children and, 159 BFP-STS and, 136

Hemolytic uremic syndrome (HUS), 24,131

Heparin, 187-8, 190,401-402 aPL and, 228 catastrophic APS and, 452 endothelial cells and, 301 HIT and, 23, 187,226-7,412 IVF and, 432 murine models and, 383 pregnancy and, 399-400 thrombocytopenia and, 410-2

Hepatitis aCL and, 137 aPL and, 139 infectious aPL and, 138 other hemocytopenias and, 27

Hernandez, D., 71 HERS study, 439-40 Het Nationaal Reumafonds, 314 Hisashi, K., 85-6 Histidine, 293-4 Histocompatibility complex, 348,

356 case study, 349-50 family studies, 350-1 MHC-DQBl alleles and, 353-4 PAPS and, 351-5 SLE and, 353

Histones, 145 HIT (heparin-induced

thrombocytopenia and thrombosis), 23

aPL and, 226-7 pregnancy and, 187 thrombocytopenia and, 412

HIV,9 aCL and, 136 aPL and, 137, 139, 151 APS in children and, 156 other hemocytopenias and, 27 pulmonary hypertension and,

100 thrombocytopenia and, 454

HLA (human leukocyte antigen), 149-150

atherosclerosis and, 167 classes, 15 DR4,8

Hojnik, M., 34 Hokko, S., 359 Homocystinuria, 14

Hopkins lupus cohort, 176 Horbach, D. A., 11, 13,248-9 Hormone replacement therapy,

439-41 HTLV-l, 10, 138 Hughes, G. R. V., 71, 77,111,393,

419-26 APS description and, 3-6 clinician's view of, 105-10

Hughes ears, 107 Hughes Syndrome

history of, 3-6 thrombocytopenia and, 408-18 see also Antiphospholipid

syndrome Human leukocyte antigen (HLA),

149-50 atherosclerosis and, 167 classes, 15 DR4,8

Hunt, B. J., 96-104, 137,449-56 HUS (hemolytic uremic

syndrome), 24,131 HUVEC

eicosanoids and, 341 endothelial cells and, 343 tissue factor and, 319-20

Hyalinization, 63 Hydralazine, 144-5, 148-9 Hydroperoxides, 337, 339 Hydroxychloroquine,392 Hyperhomocysteinemia,453 Hypermetabolism, 52 Hyperpigmentation, 63 Hyperplasia, 275 Hypertension

pulmonary, 96-101 renal artery lesions and, 71

Hypoadrenalism, 160 Hypopituitarism, 450 Hypothrombinemia,218

Idiopathic thrombocytopenic purpura (ITP), 22, 161

aPL and, 22-3 thrombocytopenia and, 410

Idiotypic network, 381-2 Immunofluorescence, 76 Immunoglobulin A (IgA), 8

aCL testing and, 206, 210 antibodies and, 116 APS pathology and, 264 atherosclerosis and, 167 cognitive dysfunction and, 49 glycoprotein and, 241-2 histocompatibility complex

and, 351 infectious aPL and, 137 infertility and, 188-9 pulmonary hypertension and,

99 skin manifestations and, 63 thrombocytopenia and, 21 tissue factor and, 319

Immunoglobulin G (IgG), 249, 376 aCL testing and, 205-6, 209 AIHA and, 24

Index

antibodies and, 116, 311-12 aPL and, 137,227,358-9,361,

367-9 APS in children and, 156-8, 162 APS pathology and, 264 aPT and, 248 atherosclerosis and, 167 catastrophic APS and, 132 cerebral ischemia and, 46 CNS and, 119 cognitive dysfunction and, 49 drug-induced aPL and, 144,

146-7,150-1 eicosanoids and, 339, 341 endothelial cells and, 300,

302-3,343 eye and, 90 genetics and, 149 glycoprotein and, 239-42 histocompatibility complex

and, 350-51 infertility and, 188-9 LA and, 222 measurement and, 10 MRL/lpr models and, 380-1 neuroimaging and, 54 placental pathology and, 286 pregnancy and, 183-5, 282, 398,

403 psychosis and, 50 pulmonary hypertension and,

99 renal pathology and, 274 skin manifestations and, 63 SNHL and, 86 thrombocytopenia and, 21,

410,412 thrombosis and, 13, 293-5, 392 thromboxane and, 341-2 tissue factor and, 318-20 TTP and, 26

Immunoglobulin M (IgM), 249 aCL testing and, 206, 210 AIHA and, 23-4 angioplasty and, 38 antibodies and, 116 aPL and, 137,227,358,361-2,

365-6,368 APS in children and, 156, 162 APS pathology and, 264 atherosclerosis and, 167 drug-induced aPL and, 145-6,

151 endothelial cells and, 301, 343 genetics and, 149 glycoprotein and, 240-1 histocompatibility complex

and, 350-1 infertility and, 188-9 LA and, 222 measurement and, 10 MRL/lpr models and, 380 pregnancy and, 183-5,398 psychosis and, 50 pulmonary hypertension and,

99 skin manifestations and, 63

Index

SLE and, 15 SNHL and, 85-6 thrombosis and, 295 tissue factor and, 318-20

Incstar Corp., 209 Infective endocarditis, 33-6 Infertility, 188-91

aPL and, 427-30 autoimmunity and, 426 IVF and, 426-32

Inflammatory bowel disease, 67 Ingram, S. B., 127 Inhibitor caspase-activated Dnase

(lCAD),373 Inhibitors, 215-16, 218 Inouey, Y., 83 INOV A Diagnostics, Inc., 209 Instituto Nacional de Cardiologia

Ignacio Chavez, 89 Interferon, 132 Interleukin, 167,302

apoptosis and, 372 fibrinolysis and, 325 MRL/lpr models and, 380 murine models and, 385

International Antiphospholipid Symposium, 179

International Normalized Ratio (lNR), 392-4, 400, 415

International Symposium on Antiphospholipid

Antibodies, 4, 210 Intracardiac thrombus, 38 Intracellular adhesion molecule

(ICAM),343 Intrarenal vascular lesions, 72-7 Intrauterine growth restriction

(IUGR), 281, 398, 401-2 Intravenous immune globulin

(IVIG), 187, 190 In vitro fertilization (lVF), 426-32

IVF-ET,188-91 Ischemia, 106-7

APS in children and, 160-1 cerebral, 4, 9, 44-6, 48, 197-200 cortical renal, 76-7 CVI,197

Isenberg, D., 358-71 Isoniazid, 144 Italian aPL Registry, 13, 21, 413 Italian Cooperative Group, 25 ITP (idiopathic thrombocytopenic

purpura), 22,161 aPL and, 22-3 thrombocytopenia and, 410

IVIG (intravenous immune globulin), 187, 190

Jamaican neuropathy, 138 Jerne's theory, 381 Jick, H., 434 Joints, 109 Joseph, J. E., 342, 411 Jouhikainen, T., 13 Julkunen, H. A., 438 Juvenile chronic arthritis OCAl,

157

Kaider, B. D., 429 Kalashnikova,1. A., 120 Kant, K. S., 71 Kaolin

clotting time (KCT) and, 217-9, 231

LA measurement and, 222 Kaplan-Meier curves, 420 Kataoka, H., 83 Kattwinkel, N., 39 Kay, G. G., 48 Kearon, c., 12 Kemeter, P., 431 Keratinocyte cell lines, 359 Kerr, J. F. R., 372 Kessel, A., 82-8 Khamashta, M. A., 3-6, lOS, 131,

391-6,415 Kidney disease, 109

cortical renal ischemia and, 76-7

ESRD and, 77 glomerular capillary

thrombosis and, 71 renal artery lesions and, 70-1 renal transplantation and, 77-9 renal vein thrombosis and, 77 thrombotic micro angiopathy

and,72-5 Kim, C. H., 429, 431-2 Kincaid-Smith, P., 72 Kininogens, 226, 256 Kittner, S., 12 Klebsiella, 422-3 Kleihauer-Betke stain, 182 Knight, R. J., 78 Koike, T., 238-44 Kontiainin, S., 156 Kornberg, A., 320 Kowalik, A., 430 Kratz, C., 156 Krause, I., 379-88 Kreidstein, S., 440 Kringle domains, 246, 327-8, 331 Krnic-Barrie, S., 46 Kunitz-type plasma proteinase, 318 Kutteh, W. H., 185, 189,255-6,

429,432

LA, see Lupus anticoagulant LAC, 162, 190 Lahita, R. G., 167-71 Lakasing, 1., 397-407 Lasne, D., 23 Laurell, A. B., 6, 136 Lavalle, c., 52 LDL (low density lipoprotein), 13,

37 atherosclerosis and, 168, 172-7 fibrinolysis and, 328, 330-1

Leach, I. A., 39 Leach, R. M., 419-26 Leaflets, 33-4 Lecithin, 135 Lee, S. 1., 136 Legionnaires disease, 136 Leprosy, 136, 139

Leroy, V., 138 Lesions

aCL testing and, 211 intrarenal vascular, 72-7 neuroimaging and, 53-4 pseudo-vasculitic,65 renal artery, 70-1 renovascular, 273 skin biopsies and, 67 treatment and, 68

Le Tonqueze, M., 300 Leukocytes

469

atherosclerosis and, 167 endothelial cells and, 302, 343 fibrinolysis and, 325 histocompatibility complex

and,351 Leukocytoclastic vasculitis, 131 Leung, W-H, 39 Levine, S. R., 43-58 1FT (liver function test), 108 Liano, F., 77 Libman-Sacks endocarditis, 33-4,

381,421 Lidegaard, 0., 435-6 Lie, J. T., 263 Lipoproteins, 21 Livedo,S, 107 Livedoid vasculitis, 61-3 Livedo racemosa, 60 Livedo reticularis, 4, 9, 454

aCL testing and, 211 APS in children and, 159 CNSand,120 cognitive dysfunction and, 48 skin manifestations and, 59-60,

67 transverse myelopathy and, 52

Liver function test and, 108 STS and, 135 thrombosis,S

Lockshin, M. D., 459-62 Loeliger, A., 247 Loizou, Sozos, 5 Lopez, 1. R., 10 Lopez-Pedrera, c., 317-24 Lopez-Sotol, A., 116 Lousiville APL Diagnostics, Inc.,

210 Low-molecular-weight heparins

(LMWH), 400, 402 Lp(a), 328, 330-2 Luchi, M. E., 99 Lupus

APS and, 3-4 contraceptives and, 439 critical illness and, 419 syphilis tests and, 5-6 see also SLE

Lupus anticoagulant (LA), 12, 149, 459

aCL and, 136-7,205-8,210-11 AlHA and, 23 antibodies and, 312 aPL and, 139, 144-8, ISO, 254,

309-10

470

Lupus anticoagulant (LA) - cant. APS in children and, 155,

157-8,162-3 APS pathology and, 264, 268,

271, 273, 277 aPT and, 245, 248 aPTT and, 216-17 autoantibodies and, 257-8 BFP-STS and, 136 cerebral ischemia and, 46 cofactor phenemenon and, 247 cognitive dysfunction and, 48 critical illness and, 419-21 detection approach to, 222 DRVVT and, 218-20 eicosanoids and, 339-40 endothelial cells and, 299 glomerular capillary

thrombosis and, 71 glycoprotein dependent, 228-30 histocompatibility complex

and,350-1 infertility and, 188-90,427,429 inhibitor and, 215-16 ITP and, 22 KCT and, 217-18 measurement and, 214-24 mechanistic/diagnostic

considerations of, 225-37

MRL/lpr models and, 381 neuroimaging and, 54 other venoms and, 220-1 PAPS and, 111-26 placental pathology and, 282-3 platelet neutralization and, 217 preanalytical variables and, 216 pregnancy and, 183-6,282 protein C and, 307 prothrombin time and, 218 psychosis and, 50 quality control and, 221-2 renal pathology and, 273 renal vein thrombosis and, 77 screening and, 215-21 steroids and, 401 tests and, 214-15 thrombocytopenia and, 20-21,

408-9,412 thrombosis and, 13,292-3,295,

392 thromboxane and, 341 tissue factor and, 319

Lyme disease, 136 Lymphopenia, 15 Lysyl,327

MaCabe, B. F., 82 McCarty, G. A., 10,263-80 Machin, S. J., 214-24 Mackie, I. J., 214-24 Mackworth-Young, C., 5,111-26 MAC (membrane attack

complex), 132 McNeil, H. P., 240 Macroglobulinemia, 63 Macrophages, 167-9

Male, C., 163 Mallory's phosphotungstic acid

hematoxylin (PTAH), 264 Mann-Whitney tests, 420 Martinelli, I., 434, 438 Martini, A., 163 Martinuzzo, M., 337-47 Masson's trichrome, 264 Matsuura, E., 37 Mayot, D., 84 MCTD (mixed connective tissue

disease),98-9 Mediterranean spotted fever, 136 Mee-Ling Boey, 5 Memory loss, 105 Meniere's disease, 84 Menon, S., 49 Merkel, P., 14 Meroni, P. L., 299-306 Merry, P., 89 MES (micro-embolic signals),

197-200 Methlyenetetrahydrofolate

reductase, 14 MHC Class II associations, 351-6 Microangiopathy, 24-5 Microemboli, 195-201 Microthrombotic occlusive

disease, 45 Microvesicles, 411-12 Migraines, 5-6, 9, 50-1, 106

APS in children and, 159 PAPS and, 119

Mikdashi, J. A., 48 Mini Mental Status Examination,

47,49 Mitral valve, 32, 34-5, 44 Mixed connective tissue disease

(MCTD).98-9 Mohr, C. F., 136 Mok, C. c., 440 Molina, J. F., 10 Mondragon-Ramirez, G., 78 Montecucco, C., 20-31 Moore, J. E., 136 Mortality, 13

aPL and, 37 catastrophic APS and, 130-1 see also Pregnancy loss

Morton, K. E., 38 M Phospholipid (MPL), 207 MRI (magnetic resonance

imaging), 53 catastrophic APS and, 130 CNS and, 119-20 micro emboli and, 195-6 neuroimaging and, 54 Sneddon's syndrome and, 48

MRL/lpr model, 379 MTHFR mutation, 452-3 Mujic, F., 15 Multiple sclerosis, 9 Murine monoclonal antibodies

(MOAB), 228, 231 Murmurs, 34-5 Murphy, J. J., 39 Mycoplasma, 136

Myelitis, 9, 54 Myelopathy, 5, 9, 52 Myocardial infarction, 36-8

APS in children and, 160 atherosclerosis and, 176

Myointimal cells, 263

Naarendorp, M., 53, 86 N-acetyl transferase, 145 Nailfold infarcts, 107 Najmey, S. S., 115

Index

National Committee for Clinical Laboratory Standards (NCCLS),207

National External Quality Assessment Scheme (NEQAS),222

National Institute for Biological Standards and Control (NIBSC),221

Navarrete, M. G., 43-58 Necrosis, 63, 67

apoptosis and, 372 other hemocytopenias and, 26

Necrotizing vasculitis, 61-3 Nelson-Piercy, C., 397-407 Nencini, P., 11-12 N eparin, 240 Nephrotic syndrome, 10 Nesher, G., 32 Netherlands Heart Foundation,

314 Neuroimaging, 53-4

see also MRI Neurological disease, 4 Neutropenia, 15 Neutrophils,26 Neuwelt, C. M., 131 N -Glycosylation sites, 238 Nilsson, I. M., 6, 136 Nip, M. M., 189,427 Nochy, D., 77 Nodules, 65 Nojima, J., 412 Nurse's Health Study, 440

Occlusions catastrophic APS and, 127-34 cortical renal ischemia and,

76-7 eye and, 89 renal artery lesions and, 71 skin manifestations and, 63

Ocular syndromes, 53 Oliveri, 0., 432-3 Oosting, J. D., 247 Opsonization, 167 Oral contraceptives, see

Contraceptives Organon Teknika, 231 Orthopnea, 34 Ostuni, P. A., 71 Ovalbumin, 383 Oxidative-modification

hypothesis, 172 Oxidized micro titer plates, 208 Oxyuranus scutellatus, 220

Index

Palmar erythema, 65, 67 Palosuo, T., 249 Pangborn, M. c., 135 Panniculitis, 269 Panzer, S., 115 PAPS, see Primary

antiphospholipid syndrome

Papules, 65 Parke, A. L., 281~9 Parvovirus, 138~9 Patel, Bupendra, 5 PCA (procoagulant activity),

317~20

Peaceman, A. M., 342 Peddi, V. R., 14 Pelvic inflammatory disease, 190 PE (phosphatidylethanolamine),

248,256,264,269,272~3

APC resistance and, 313 renal pathology and, 274

Peptides, 384 Perez, R. E., 76 Pericellular proteolysis, 325~ 7 Peripheral blood lymphocytes

(PBL), 366~ 7 Perivasculitis, 273 Petechial purpura, 63 Petitti, D., 436 PET scan, 52, 54 Petri, M., 99, 426~46 Phenytoin, 144~5, 148~9 Phosphatidylcholine, 23, 138 Phosphatidylethanolamine (PE),

248,256,264,269,272~3

APC resistance and, 313 renal pathology and, 274

Phosphatidylglycerol (PG), 206, 358

Phosphatidylinositol, 358 Phosphatidylserine (PS), 46, 138,

206,210,341,358~9

PH (pulmonary hypertension), 5 APS and, 97 ~8 managament of, 100~1 primary, 96, 98~ 10 1

Physicians Health Study, 226 Pierangeli, S. S., 135~43, 205~13,

302,380 Piette, J. C., IS, 96~ 104, 117 Placental pathology, 281~3

pathogenesis and, 286~7 University of Connecticut and,

285~6

Platelets, 460 antibodies and, 114~15 aPL and, 227 catastrophic APS and, 131, 133 endothelial cells and, 303 fibrinolysis and, 325 infectious aPL and, 137 KCT and, 217~18 LA measurement and, 216 neutralization and, 217 plugs and, 307 thrombocytopenia and, 21~2,

409~15

thrombosis mechanism and, 290~8

thromboxane and, 341~2 venom and, 215, 218~21

Pneumonitis, 272 Polyarteritis nodosa, 63 Polymyxin B, 222, 231 Porter, R. Flint, 179~94 Potzsch, B., 312 Poulter, N. R., 434 Poux, J. M., 71 PPH (primary pulmonary

hypertension), 96, 98~101 Pre-eclampsia, 184~6, 398 Pre-embryonic period, 180 Pregnancy

anticoagulation and, 4 aPL and, 14, 161~2 APS and, 3, 8, 397 ~407 chorea and, 51 clinical criteria and, 9 demographics and, 8 drugs and, 187~8 infertility and, 188~91, 426~32 LA measurement and, 222 MRL/lpr models and, 381 murine models and, 383 oral contraceptives and, 432~41 PAPS and, 120~1 placental pathology and, 281~9 thrombosis and, 392 uterine changes and, 282

Pregnancy loss, 451~3, 460~1 aCL testing and, 21 o~ 11 APS and, 186~8, 397~8 classification and, 179~80 complications and, 184~5 epidemiology and, 180~ I patient evaluation and, 181~4 types of, 183~4

Prekallikrein, 256 Primaryantiphospholipid

syndrome (PAPS), 122 antibody specificity and,

113~16

cardiovascular system and, 119 children and, 162 classification criteria for,

116~17

clinical presentation and, 117~21

clinical spectrum and, 112~13 CNS and, 119~20 defining, 111~12 drug-induced aPL and, 144 histocompatibility complex

and,349,354~5 LA and, 225 MES and, 198 MHC Class II and, 351 ~5 monoclonal aPL and, 367 recurrent abortion and, 120~ 1 SLE-APS differentiation and,

113 thrombocytopenia and, 121 thrombosis and, 118~19 tissue factor and, 318~19

471

treatment of, 121 see also Antiphospholipid

syndrome (APS) Procainamide, 144

drug-induced aPL and, 147~8, 150~1

secondary APS and, 15 Procoagulant activity (PCA),

317~20

Prodromal symptoms, 48 Propranolol, 149 Propylthiouracil, 144 Prosser-White lecture,s Prostacyclin, 226, 299

endothelial cells and, 302~3, 343~4

fibrinolysis and, 325 Prostaglandin, 343 Proteins, 307, 314, 460

acquired deficiencies and, 313 antibodies and, lIS antithrombomodulin

antibodies and, 311~12 APC resistance and, 312~ 13 aPL specificity and, 309~10 apoptosis and, 373~5 aPT and, 249 atherosclerosis and, 167 autoantibodies and, 257~8 biological surfaces and, 327~9 C, 307~10, 314, 460 contraceptives and, 432~3, 438 critical illness and, 420 fibrinolysis and, 333 GFAP,264 HDL,169 HUVEC and, 300~4 immunoassays and, 256 Kunitz-type,318 LA and, 226 LDL,168 PAPS and, 118 prothrombin and, 245~6 S, 309~1O, 313 thrombin paradox and, 31O~1 thrombosis and, 291~2, 454 see also Glycoprotein

Proteinuria, 72 Proteoglycan,227 Prothrombin, 245~7, 251

antibodies and, 114, 247~50 aPL specificity and, 309~10 autoantibodies and, 257 contraceptives and, 438 infectious aPL and, 138 pregnancy and, 403 PT and, 218 PTT and, 90, 206, 231

Prothrombotic diathesis, 299 Pro urokinase, 325~6 P-selectin, 291, 411 Pseudomonas, 422 Pseudonaja textilis, 220 Pseudo thrombocytopenia , 23 Pseudo-vasculitic lesions, 65 Psychosis, 50 PT (prothrombin time), 218

472

PTT (partial thromboplastin time), 90, 206, 231

Pulmonary hypertenstion (PH), 5 APS and, 97-8 managament of, 100-1 primary, 96, 98-10 1

Pulmonary thromboembolism (PTE), 272

Pustules, 65 Puurunen, M., 249 Pyoderma gangrenosum, 454

Q fever, 136, 205, 209 Quinidine, 145, 149

Radhakrishnan, J., 78 Rahman, A., 358-71 Rapaport, S. I., 225, 247 Raschi, E., 299-306 Ravelli, A., 158, 163 Raynaud's phenomenon, 4, 63,

98-9, 161 Reactive oxygen species (ROS),

359 Recurrent abortion, 120-1 Regurgitation, 33-4 Renal artery lesions, 70-1 Renal scintigraphy, 71 Renal transplantation, 77-9 Renal vein thrombosis, 77 Reproductive autoimmune

failure syndrome, 188 Reverter, J. c., 290-8 Reyes, P. A., 89-95 Rheumatic fever, 33-4 Rheumatoid arthritis, 63

BFP-STS and, 136 catastrophic APS and, 128 CPZ-induced aPL and, 146

Rioux, J. D., 368 Rorastiero, R., 337-47 Rose Bengal, 232 Rosenberg, R. D., 450 Rosendaal, F. R., 14,437 Rosing, J., 435 Rossi, E., 71 Rote, N. S., 341 Roubey, R. A. S., 229, 239, 253-60,

320 rTP A (recombinant tissue

plasminogen activator), 68 Ruckenstein, M. J., 83 Ruiz-Arguelles, G. J., 52 Russell's viper venom (RVV),

218-19

St. Thomas' Hospital, 14,249, 401-2,452

Saleh, A. A., 439 Sammaritano, 1. R., 144-54 Saunders, M., 136 Scarabin, P. Y., 439 scFv,382 Schirmer test, 4 Schizophrenia, 145 Schmidt, R., 12,49 Schneider, M., 195-201

Schoning, M., 161 Schulman, S., 12 Schved, J. F., 11 Schwartz, M., 50 Schwartz, R., 372 Schwartz, S., 436 SCID (severe combined

immunodeficient) disorder, 83

Sclerodactyly, 98 Scleroderma, 63, 98, 128 SD (sudden deafness), 82 Seizures, 51, 106

treatment and, 54 SELENA study, 441 Sensorineural hearing loss

(SNHL),52-3 animal models for, 83 autoimmune diseases and, 82-3 immunological profile in, 84 primary APS and, 85 secondary APS and, 86

Serological test for syphilis (STS), 5-6,135-6

aCL testing and, 206 histocompatibility complex

and, 349-50, 354 LA and, 225

Severe combined immunodeficient (SCID) disorder, 83

Shah, N. M., 15,46 Sharp, P. A., 84 Sheehan's syndrome, 450 Sheng, Y., 240 Sher, G., 432 Sheridan-Pereira, M., 162 Shi, W., 293 Shigeta, H., 52 Shin, S., 84 Shoenfeld, Y., 6,342,379-88 Sickle cell anemia, 26 Siebler, M., 195-201 Silver, R. K., 162 Silver, R. M., 15, 179-94 Silvestrini, M., 51 Simanitov, R., 132 Simioni, P., 12 Simplastin,231 Singer, H. S., 157 SIRS (systemic inflammatory

response syndrome), 132-3

Sjogren's syndrome, 4, 63 BFP-STS and, 136 histocompatibility complex

and, 350, 355 SNHL and, 82, 86

Skin manifestations, 454, 461 cutaneous gangrene, 63 different organ involvement

ant, 67 lesion biopsies and, 66-7 livedo reticularis and, 59-60 necrosis, 63 pseudo-vaculitic lesions, 65 Sneddon's syndrome and, 61

Index

subungual hemorrhages, 66 thrombophlebitis, 63-4 treatment and, 68 ulcers, 61 vasculitis, 61-3 see also Livedo reticularis

SLE, see Systemic lupus erythematosus

Sletnes, K. E., 37 Smallpox, 136 Smoking

atherosclerosis and, 174 myocardial infarction and, 38

Sneddon's syndrome, 48, 460 micro emboli imaging and, 197 skin and, 61, 107 see also Livedo reticularis

SNHL (sensorineural hearing loss)

animal models for, 83 autoimmune diseases and, 82-3 immunological profile in, 84 primary APS and, 85 secondary APS and, 86

Somatic hypermutation, 361-2 Soulier, J. P., 6 Specker, C., 46, 195-201 Spiera, H., 53, 86 Spinal cord, 106 Spitzer, W.O., 434 Splenomegaly, 9 ssDNA,145 Staphylococcus aureus, 422 Stephansson, E. A., 354 Stern, J. J., 430 Steroids, 4, 401 Stewart, M. W., 210 Streptococcus, 182 Streptokinase, 131 Strokes, 4-6, 106

aPL and, 37 APS in children and, 160, 162 atherosclerosis and, 176 catastrophic APS and, 131 cerebral ischemia and, 44 cognitive dysfunction and, 48 migraines and, 51

STS (serological test for syphilis), 5-6,135-6

aCL testing and, 206 histocompatibility complex

and, 349-50, 354 LA and, 225

Subungual hemorrhages, 66 Suissa, S., 434 Superficial thrombophlebitis, 63 Sushi domains, 232, 238, 240 SVC (superior vena cava)

thrombosis, 158 Syndrome of the Black Swan, The

(Harris), 179 Syphilis, 5-6, 9

aCL and, 136, 206 glycoprotein and, 241 histocompatibility complex

and, 349 infectious aPL and, 137-9

Index

LA and, 225 serological test for, 5-6, 135-6,

206,225,349-50,354 Systemic lupus erythematosus

(SLE), 4-5, 8,17,161,249, 376,460-2

aCL and, 136-7,205,209 AIHAand,23 APC resistance and, 312-13 aPL and, 11, 14, 147 apoptosis and, 372, 375-7 APS in children and, 155,

157-9, 162 APS pathology and, 195,268,276 aPT and, 248-9 atherosclerosis and, 168-9, 172,

174, 176 BFP-STS and, 136 cardiomyopathy and, 39 catastrophic APS and, 127-34 cognitive dysfunction and, 49 coronary artery disease and,

37-8 critical illness and, 419-21,

423-5 drug-induced aPL and, 144-5,

149 eye and, 90 fibrinolysis and, 329, 332 glycoprotein and, 241 histocompatibility complex and,

350-1,353,355 hormone replacement and,

440-1 infectious aPL and, 137 ITP and, 22 kidney disease and, 70-1 LA and, 20, 214, 225 measurement and, 10 MES and, 198-200 monoclonal aPL and, 358, 368 MRLllpr models and, 381 neuroimaging and, 53-4 PAPS and, 15, 111-26 placental pathology and, 282-3,

285,287 pregnancy and, 186, 397 procainamide-induced aPL

and, 147-8 psychosis and, 50 pulmonary hypertension and,

98-9 renal pathology and, 273 renal transplantation and, 77-9 renal vein thrombosis and, 77 seizures and, 51 skin manifestations and, 63 SNHL and, 83-4, 86 thrombocytopenia and, 20-1 thrombosis and, 13, 392, 394 tissue factor and, 318 TMA and, 72-5, 263, 269 transverse myelopathy and, 52 TTP and, 25-6 uterine changes and, 282 valvular disease and, 32-6 vasculitis and, 61-3

Systemic inflammatory response syndrome (SIRS), 132-3

Systemic necrotizing vasculitis, 128

TAFI, 308, 311 Taipan venom, 220, 222 TAIYF,369 Takao Koike, 4, 6 Tassies, D., 290-8 Taylor, P. V., 427 TCD (trans cranial Doppler

sonography), 197-200 T cells, 376

atherosclerosis and, 168 bone marrow transplantation

and,382-3,385 immunodulation and, 383

Telangiectasia, 63, 98 Textarin, 220-2 TGA (transient global amnesia),

53 Thiazide, 128 Thickening, 32-3 Thrombin

infectious aPL and, 138 paradox, 310-11

Thrombocytopenia, 4-5, 9, 13, 228,342,395,454,461

aCL testing and, 210 aPL and, 21-3 APS in children and, 155, 161 APS pathology and, 272 blood and, 108 catastrophic APS and, 130,452 clinical features of, 21 heparin-induced,410-12 histocompatibility complex

and,350 management of, 412-14 MRLllpr models and, 379, 381 PAPS and, 121 pathophysiology of, 408-10 prevalence in APS and, 20-1 thrombosis and, 414-15 transverse myelopathy and, 52

Thrombomodulin, 308, 310, 312 fibrinolysis and, 325

Thrombophilic diathesis, 299 Thrombophlebitis, 63 Thromboplastin, 218 Thrombosis, 3, 5, 228, 459-60

aCL testing and, 205-6, 211 antibodies and, 116 anticoagulants and, 9-10 aPL and, 11, 147, 151,226-7,392 APS in children and, 155-6,

158-62 arterial, 3-4, 11, 450-1 atherosclerosis and, 175-6 autoantibodies and, 257 cardiovascular, 37-8 catastrophic APS and, 127-34 cerebral disease and, 43, 45-6 clinical criteria and, 9 contraceptives and, 432-8 cortical renal ischemia and, 76-7 demographics and, 8

ECAT and, 231 eye and, 93-4 fibrinolysis and, 330-1 glomerular capillary, 71 hormone replacement and,

439-41 infectious aPL and, 137 LA and, 13, 225, 232-4 management of, 391-6 measurement and, 10 mechanism of, 290-8 MRLllpr models and, 380 murine models and, 383 PAPS and, 118-19

473

placental pathology and, 285 pregnancy and, 186,397,452-3 psychosis and, 50 pulmonary hypertension and,

99 renal vein, 77 risk factors and, 13-14 skin manifestations and, 67 SVC, 158 tissue factor and, 317, 320 transverse myelopathy and, 52 treatment and, 54-5 valvular disease and, 34 vascular, 449 venous, 3-4, 47, 450

Thrombotic micro angiopathy (TMA), 72-5, 263, 268-9, 271

APS pathology and, 275-6 renal pathology and, 273 venous, 272

Thrombotic thrombocytopenic purpura (TTP), 24-5

aPL and, 25-6 catastrophic APS and, 131 tissue factor and, 318

Thromboxane, 338, 341-2, 344, 411,413

endothelial cells and, 343 murine models and, 383

Thymus, 107-8 TIA (transient ischemic attacks),

106 Tietjen, G. E., 51, 54 Tincani, A., 240, 299-306 Tinnitus, 82 Tissue factor, 317-18

induction mechanisms and, 320-1

in vitro studies and, 319-20 tissue factor pathway inhibitor

(TFPI),318-19 VF behavior of, 318-19

Tissue- type plasminogen activator (t-PA), 325-6, 328-9

TMA (thrombotic microangiopathy),72-5, 263,268-9,271

APS pathology and, 275-6 renal pathology and, 273 venous, 272

Toubi, E., 53, 82-8

474

Tourbah, A., 48 Tourette's syndrome, 157 TRAELDT region, 353-5 Transcranial Doppler sonography

(TCD),197-200 Transient global amnesia (TGA),

53 Transient ischemic attacks (TiA),

106 Transnational Study, 436 Transverse myelitis, 161

APS in children and, 159 treatment and, 54

Transverse myelopathy, 52 aCL testing and, 211

Treponema palladium, 135 Triplett, D. A., 149 Tri-sodium citrate, 216 TSP (tropical spastic paraparesis),

138 Tsutsumi, A., 10,238-44 TTP (thrombotic

thrombocytopenic purpura), 24-5

aPL and, 25-6 catastrophic APS and, 131 tissue factor and, 318

Tuberculosis, 136 Tucker. L. B., 155-66 Tumiati, B., 86 Tumor necrosis factor (TNF), 318,

325 apoptosis and, 372 atherosclerosis and, 167 catastrophic APS and, 132-3

TXA,303

Ulcerative colitis, 67 Ulcers, 61 Ulcova-Gallova, Z., 429 Ultrasound, 197-200 University of Connecticut, 285-6 Unstable angina, 36 u-PAR receptor, 325

U. S. Preventive Services Task Force, 191

Vaarala, 0., 36, 38, 172-8, 249, 392 Vaidya, S., 78 Valproic acid, 149 Valve thrombi, 16 Valvular disease

cerebral ischemia and, 44-5 clinical manifestations and,

34-6 pathogenesis and, 34 thickening and, 32-3 vegetation and, 33-4

Valvulopathy,32-3 Vandenbrouke, J. P., 437 Varicella zoster virus, 136 Vascular cell adhesion molecule

(VCAM),343 Vascular disease, 108

catastrophic APS and, 127-34 fibrinolysis and, 325-7

Vascular endothelial growth factor (VEGF), 96

Vascular pathology, 263, 277 cardiovascular, 271 cerebrovascular, 274-5 characteristic APS findings,

264-8 cutaneous, 268-71 methods of, 264 other organs, 276 pulmonary, 272-3 renal, 273-4

Vasculitis, 61-3, 263, 269 placental pathology and, 285

VCAM-l,302 Vegetations, 33-4 VEGF (vascular endothelial

growth factor), 96 Velasco, F., 317-24 Venereal Disease Research

Laboratory (VDRL), 90, 136,349

Index

Venom, 215, 218-22, 231 Venous thromboembolism (VTE),

11-13 Venous thrombosis, 3-4, 47, 450 Vermylen, J., 225-37 Verro, P., 46 Verrot, D., 51, 119 Vertigo, 107

SNHL and, 82 Vianna, J. L., 15, 113 Viard, J. P., 116 Vierhoeffvan Gieson (VVG), 264 Vincent, R., 111-26 Viral peptide, 139 Vitamin D, 187 Vitamin K, 229, 308

tissue factor and, 321 von Willebrand factor, 132, 325 VTE,11-13

Wahl, D. G., 12-13 Walnut Creek Contract Drug

Study, 439 Walport, Mark,S Warfarin, 37, 110,393-4

catastrophic APS and, 131 pregnancy and, 399-401 thrombocytopenia and, 415

Wasserman reaction,S, 135-6 White atrophy, 63 Willems, G. M., 229 Williams, F. M. K., 419-26 Wilson, W. A., 348-57 Workshop on Diagnostic Criteria,

461 Wu, J. R., 248 Wyllie, A. H., 372

Yasuda, M., 185 Yetman, D. L., 255-6 Yron,I.,430

Zelzer, B., 48 Ziporen, L., 34