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5 Treating Relapses, Refractory Disease and Other Agents

5 Treating Relapses, Refractory Disease and Other Agents

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430



L. Guillevin



References

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French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 90(1):19–27

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Nephrol 7(2):240–247

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relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis.

Arthritis Care Res (Hoboken) 62(8):1166–1173

12. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS et al (2010) Rituximab

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or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med

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14. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J et al (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic

autoantibodies. N Engl J Med 349(1):36–44

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35 Treatment of ANCA-Associated Vasculitides



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long-term follow-up. Ann Rheum Dis 71(6):955–960

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28. Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L et al (2008) Intravenous

immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two

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Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18(7):2180–2188



Index



A

Abatacept, 60, 286, 411

Adalimumab, 94, 115, 271, 286, 305, 413

Adhesion molecules, 6, 7, 120, 148, 149, 204

Alemtuzumab, 115, 286, 412

Alfa1-antitrypsin gene variants, 44

Alport syndrome, 198

Amaurosis fugax, 84, 86

Amyloid angiopathy, 253

Amyloid-β related angiitis (ABRA), 250, 254

Anakinra, 150, 182, 286

ANCA-associated vasculitides (AAV), 9, 10,

12, 21, 23, 24, 32–35, 38, 44, 45, 47,

48, 110–116, 125, 141–153, 160,

162–165, 167, 168, 237, 295, 303,

407–414, 425–429

Angiogenesis, 4, 6, 373

Angiography, 26, 30, 90, 180, 184, 194, 246,

247, 249–253, 263, 266, 303, 350, 354,

369, 381, 391, 392, 396, 398

Animal models, 9–12, 45, 58, 71, 111, 148,

150, 198

Anisosphygmia, 56

Anterior ischemic optic neuropathy (AION),

84, 85, 87

Anti-angiogenic molecules, 4

Anti-annexin V antibodies, 36, 37

Anti-C1q autoantibodies, 23

Anti-centromere antibodies, 367

Anti-cyclic citrullinated peptide

antibodies, 89

Anti-double stranded (ds) DNA

antibodies, 348

Anti-endothelial cell antibodies (AECA), 36,

37, 300, 326, 346, 349, 350, 408



Anti-ferritin antibodies, 36, 37

Anti-ganglioside antibodies, 270

Antigen-presenting cells, 104, 286

Anti-glomerular basement membrane

antibodies, 35, 114

Anti-heat shock protein 70 antibodies, 291

Anti-LAMP-2 antibodies, 34, 35, 37

Anti-neutrophil cytoplasmic autoantibodies

(ANCA), 9, 10, 12, 16, 19, 21, 22,

30–35, 38, 43–48, 110, 111, 120, 123,

130, 132, 133, 141–149, 151, 159–168,

192, 194, 200, 240, 246, 260, 263, 266,

267, 269, 293, 316, 349, 428

Antiphospholipid syndrome, 346

Anti-scl-70 antibodies, 367

Anti-sulfatide antibodies, 262

Aortic aneurysm, 23, 80, 91, 292, 391, 392,

394, 399

Aortic arch syndrome, 84

Aortitis, 19, 23, 54, 80, 86, 87, 91, 292, 295,

313, 317, 352, 388, 389, 393, 399

Aphthous stomatitis, 228

Arthralgias/Arthritis, Hives, Angioedema

syndrome, 324

Artificial neural network (ANN), 26

Aseptic meningitis, 175, 177, 180, 324, 326

Aspirin, 60, 90, 93, 181, 183, 220, 372

Audio-vestibular dysfunction, 291

Autoimmune pancreatitis (AIP), 311, 314,

318, 319

Azathioprine, 59, 94, 115, 126, 135, 163, 164,

195, 210, 214, 215, 220, 242, 243, 252,

253, 272–274, 285, 305, 319, 327, 350,

354, 355, 407, 409, 428

Azurophilic granules, 10



© Springer International Publishing Switzerland 2016

F. Dammacco et al. (eds.), Systemic Vasculitides: Current Status and

Perspectives, DOI 10.1007/978-3-319-40136-2



433



434

B

Balloon angioplasty, 60

B-cell activating factor (BAFF), 338, 408, 411

B-cell non-Hodgkin's lymphoma

(B-NHL), 338

Behỗets disease (BD), 23, 304, 398, 410,

412415

Belimumab, 115, 408, 411

Beta-4 amyloid, 248

Birdshot chorioretinopathy, 300, 302

Birmingham Vasculitis Activity Score (BVAS),

33, 125, 132, 160, 161, 164

Bortezomib, 45, 272, 319

Budd-Chiari syndrome, 230

Buerger’s disease, 362–374, 395

Bypass surgery, 372, 373



C

C1q receptors, 337

Canakinumab, 286, 328, 415

Cannabis-associated arteritis, 362, 381

Carbimazole, 271

Central nervous system vasculitis, 23

Cerebral angiography, 247, 249, 396

Cerebrovascular accident, 81, 90, 292

Chapel Hill Consensus Conference (CHCC),

16–26, 33, 35, 36, 110, 120, 122, 190,

192, 197, 207, 260, 261

Chorioretinitis, 282

Choroiditis, 282

Churg-Strauss syndrome, 6, 10, 20–22, 24, 25,

110, 130, 141, 192, 259, 261, 266,

300, 397

Circulating anti-proteinase-3 neutrophil

cytoplasmic antibodies (c-ANCA),

30–33, 114, 120, 123, 267, 291

Cochlear implantation, 295

Cogan’s syndrome, 23, 289–295

Cognitive impairment, 248

Colchicine, 195, 220, 241–243

Color duplex ultrasound (CDUS),

387–390, 394

Computed tomography angiography (CTA),

26, 391–394, 396, 398, 399, 401

Computerized tomography (CT), 387

Contrast angiography, 89, 90

Contrast-enhanced MRI, 247

Coronary aneurysms, 176, 180, 182, 349, 398

Coronary vasculitis, 130, 231, 349

Crescentic glomerulonephritis, 22, 35, 45,

112–115, 131

Crohn’s disease, 70–71, 300



Index

Cryocrit, 327, 336

Cryoglobulinemic vasculitis, 6, 22, 30, 237,

238, 240, 241, 274, 334, 335, 407, 410

Cryopyrin-Associated Periodic Syndromes

(CAPS), 327

Cutaneous leukocytoclastic angiitis, 233

Cutaneous small vessel vasculitis, 23, 233,

241, 243

Cyclophosphamide, 59, 94, 114, 115, 125,

135, 159, 160, 162–164, 166, 167, 181,

195, 200, 201, 210, 214, 242, 252, 269,

272–274, 319, 327, 350, 354, 355,

407–410, 426–429

Cyropyrin, 412, 415



D

Daclizumab, 412

Dapsone, 242, 243, 327

Decidual vasculitis, 219

Demyelinating polyneuropathy, 272

Digital subtraction angiography (DSA),

394–396, 398

Dihydrofolate reductase, 285

Direct acting antiviral agents, 340, 341

Disease-modifying anti-rheumatic drugs

(DMARDs), 231, 286, 355

Doppler ultrasonography, 59

Double filtration plasmapheresis, 341

DRB1*11, 99

Drug-induced ANCA-associated vasculitis, 34

Drug-induced cerebral vasculopathy, 251

Drug-induced neuropathy, 269



E

Ehlers-Danlos syndrome, 400

Elastic lamina, 57, 81–83, 92, 365, 366

Endarteritis, 364, 366

Endothelial cells (ECs), 3–5, 34–36, 81, 111,

142–144, 148, 149, 151, 175, 204, 290,

323, 337, 338, 346, 364

End-stage renal disease, 112, 162, 292

Eosinophilic granulomatosis with polyangiitis

(EGPA), 10, 21, 22, 24, 25, 33, 34, 36,

43, 110, 111, 113, 130–137, 141, 142,

161, 165, 166, 217, 260, 294, 303, 410,

415, 425–427

Eotaxin-1, 132

Ephrin family, 4

Episcleritis, 23, 112, 122, 142, 303, 324,

326, 327

Erythema nodosum, 227, 228



Index

Etanercept, 115, 271, 286, 295, 412–413

European Medicines Agency (EMA), 24, 33

algorithm, 24, 25, 191, 192

Extracellular matrix degradation, 366

Eye and ear syndrome, 289



F

Fibrinoid necrosis, 19–21, 113, 149, 199, 206,

207, 238, 248, 264, 336, 337, 425

Fibroblast Growth Factor (FGF), 183

Fibromuscular dysplasia, 191, 400, 401

Fibrotic scar, 317–319, 429

Five Factor Score (FFS), 135, 160, 161, 194,

195, 266

Fluorescein angiography (FA), 85

Fluorodeoxyglucose, 292, 396



G

Galactose-deficient IgA1 (Gd-IgA1), 205

Genital ulcers, 220, 228

Genome-wide association studies (GWAS),

33, 44, 69, 105, 142

Gevokizumab, 286

Giant cell arteritis (GCA), 6, 19–21, 36, 37,

79–95, 97–105, 246, 251, 260, 261,

265, 300, 388, 389, 391, 397, 413, 414

Glomerular basement membrane (GBM), 37,

113, 198, 337

Glucocorticoids, 7, 81, 88, 91–95, 132, 159,

164, 165, 194, 195, 210, 211, 229, 252,

253, 295, 312, 319, 327, 355, 407–409,

426

Glycosylated IgGA1, 22

Golimumab, 286

Goodpasture’s antigen, 197, 198

Goodpasture’s syndrome, 35, 125, 197

Granulomatosis with polyangiitis (GPA), 9, 10,

20–22, 24–26, 30, 33, 34, 36, 43, 44,

110, 111, 113, 119–128, 130–137, 160,

161, 164–166, 192, 218, 260, 261, 267,

274, 293, 300, 303, 408–413, 425–429



H

Half-antibody exchange, 315

Halo sign, 89, 388, 389

HCV-associated cryoglobulinemic vasculitis,

24, 410

HCV RNA, 334, 336, 337, 341

Hemorrhagic alveolitis, 126, 335

Hemorrhagic cystitis, 427

Henoch-Schönlein purpura, 25, 205, 260

Heparan sulphate, 3



435

Hepatitis B virus (HBV) reactivation,

191–194, 216, 261

HLA B35, 204

HLA-B51, 227

HLA-B*52:01, 64, 67, 69

HLA-B*67:01, 69

HLA class I genes, 98

HLA class II genes, 98

HLA-DPB1, 44, 100, 103, 104, 142, 143,

312, 351

HLA DRB1*01, 99, 204

HLA-DRB1*04, 101, 102, 104

HLA-DRB1*07, 101, 204

HLA DRB1*14, 204

HLA-DRB4, 111, 132

HLA-DRB*1501, 198

HLA-DRB*1502, 198

Hydralazine-associated MPA, 24

Hydroxychloroquine, 94, 242, 327, 350

Hypereosinophilic syndrome (HES), 134

Hypersensitivity vasculitis, 233

Hypertrophic pachymeningitis, 313

Hyperviscosity syndrome, 335

Hypocomplementemic urticarial vasculitis

(HUV), 22, 25, 30, 36, 38, 239, 260,

322, 326

Hypopyon, 112



I

Idiopathic retroperitoneal fibrosis, 315

IgA vasculitis (Henoch-Schönlein)

(IgAV), 239

IgG4, 311

IgG4-related chronic periaortitis, 313

IgG4-related disease, 267, 311–319

IgG4-related lymphoplasmacytic

cholecystitis, 313

IgG4-related neuropathy, 267

IgG4-related sclerosing cholangitis, 313

IL12p40, 71

Imaging techniques, 26, 89, 103, 394,

400, 401

Immune tolerance, 98

Immunochip, 103, 105

Inflammatory bowel diseases (IBD), 34, 43,

57, 64, 70, 71, 251, 260, 271, 282, 285,

326, 328

Inflammatory eye diseases, 295

Infliximab, 59, 94, 115, 182, 242, 271, 286,

295, 305, 412–413

Inner ear autoimmune disease, 290

Intercellular adhesion molecule-1 (ICAM-1),

6, 148, 149, 364

Interleukin-1beta (IL1β), 120, 327



436

Interleukin-6 (IL6), 58, 81, 89, 175

Interstitial keratitis, 23, 289, 291, 292

Interstitial pneumonitis, 427

Intravenous immunoglobulin (IVIG),

181–183, 214, 271

Ipililumab, 272

Iridocyclitis, 112, 282

Iritis, 85, 282

Ischemic optic neuropathy, 80

Ischemic stroke, 378–380



J

Jaccoud’s arthropathy, 324

Jaw claudication, 83, 85, 87, 93

Juvenile idiopathic arthritis (JIA), 282–285



K

Kawasaki disease (KD), 19–21, 37, 174,

181–184, 388, 397



L

Lanham criteria, 24, 134, 192

Large vessel vasculitis (LVV), 18, 19, 26, 54,

55, 58, 79, 80, 84, 87, 88, 93, 94, 295,

390, 396

Leflunomide, 59, 94, 274, 295, 428

Leukocytoclastic vasculitis, 23, 36, 113, 203,

207, 208, 233, 272, 322, 336, 338

Leukoencephalopathy, 410, 428

Leukotriene-receptors antagonists

(LTRA), 133

Levamisole, 44, 271

Limb arteritis, 378, 381

Livedo reticularis, 36, 112, 191, 237, 324,

335, 346

Lower limb arteritis, 381

Lupus vasculitis, 24, 345–350

Lysosomal-associated membrane protein 2

(LAMP2), 43, 45, 46



M

Macaroni sign, 390

Magnetic resonance angiography (MRA), 26,

30, 56, 247, 249, 252, 387, 394,

396, 398

Magnetic resonance imaging (MRI), 55, 88,

89, 92, 180, 184, 247, 249–254, 293,

317, 350, 387, 393, 394, 396, 398, 401

Marfan’s syndrome, 400

Masquerade syndrome, 282, 283



Index

Matrix metalloproteinases (MMPs), 58, 366

McDuffie syndrome, 326–327

Medium vessel vasculitis (MVV), 18, 19, 21,

30, 354, 387, 397

Memory B cells, 408–410

Ménière’s syndrome, 291

Mepolizumab, 135, 415

Mesenteric vasculitis, 131, 348, 350, 354

Mesna, 166, 167, 273, 274

Methotrexate, 59, 94, 115, 126, 135, 160, 163,

181, 195, 214, 242, 252, 255, 272–274,

285, 295, 305, 319, 354, 355, 407, 415,

428, 429

Microaneurysm, 192, 266, 302, 396

Microbubble contrast-enhanced

ultrasonography, 390

Microscopic polyangiitis (MPA), 6, 10, 19–22,

24–26, 33, 34, 36, 43, 44, 47, 110–116,

130, 134, 141, 142, 164, 165, 190, 192,

194, 218, 260, 267, 271, 274, 303, 408,

409, 414, 425–427

Migrainous vasospasm, 251

Mikulicz’s disease, 313

Minocycline, 34, 271, 272

Mixed cryoglobulinemia, 38, 269, 274,

334, 410

Modified Th2 immune-response, 315

Molecular mimicry, 34, 45, 144, 206

Mononeuritis multiplex, 112, 130, 192, 193,

216, 217, 267, 269, 272, 335, 347, 351

Mononeuropathia multiplex, 262, 263,

266–271

Motor-sensory axonopathy, 335

MPO-ANCA, 12, 21, 22, 24, 33, 34, 37, 111,

142, 145, 151, 162, 425, 428

Mucocutaneous lymph node syndrome,

19–21, 174

Multinucleated giant cells, 20–21, 57,

81–83, 365

Mycophenolate mofetil (MMF), 59, 115, 211,

242, 252, 255, 274, 285, 295, 305, 319,

327, 350, 428

Myeloperoxidase (MPO), 10–12, 21, 32, 34,

37, 43, 45–47, 111, 114, 141–147, 149,

151, 163, 164, 218



N

Naproxen, 271

Necrotizing and crescentic glomerulonephritis

(NCGN), 10, 142, 144–151, 153

Necrotizing arteritis, 19, 21, 22, 353

Necrotizing glomerulonephritis, 10–12, 30,

110, 111, 199, 352, 397



Index

Neoangiogenesis, 81, 194

Neuropilin-1 (NRP-1), 5

Neuropilin-2 (NRP-2), 5

Neuro-psycho-Behỗet disease, 226232

Neuroretinitis, 282

Neutrophil cytoplasmic antigens (NCA),

4547

Neutrophil enzymes, 32

Neutrophil extracellular traps (NETs), 45–48,

149, 151

N-methyl-D aspartate receptor, 348, 349

Non-collagenous domain 1 (NC1), 35, 197

Normocomplementemic urticarial vasculitis

(NUV), 322, 326

Notch family, 5



O

Obliterative phlebitis, 318

Occlusive vasculitis, 302, 304, 306, 347

Omalizumab, 135, 328

Optic nerve ischemia, 93

Optic neuropathy, 93, 112

Orbital pseudotumor, 313

Ormond’s disease, 313



P

Palpable purpura, 112, 130, 207, 233–236,

335, 346

Panniculitis, 346

Panuveitis, 282

Paraneoplastic vasculitic

neuropathy, 269

Pars-planitis, 282

Pathergy test, 227

Pauci-immune glomerulonephritis, 11, 45, 119

Pauci-immune vasculitis, 33

Pegylated interferon-α, 274, 340

Pentoxifylline, 242

Pentraxin-3 (PTX3), 46, 59, 111

Perinuclear ANCA (p-ANCA), 30–33, 114,

123, 132, 194, 267, 291

Perivascular sheathing, 299, 302

Phenytoin, 271

Phlebitis migrans, 367, 369

Plasma exchange, 10, 115, 126, 135, 162, 181,

195, 200, 201, 273, 274, 429

Platelet-derived growth factor, 81

Platelet endothelial cell adhesion molecule-1

(PECAM-1), 143, 144

Polyarteritis nodosa (PAN), 19, 36, 37, 134,

161, 190, 191, 216, 263, 265–266, 303,

388, 396



437

Polymyalgia rheumatica (PMR), 54, 80, 84,

86, 87

Positron emission tomography (PET), 26,

89–91, 292, 317, 387, 396–400

Posterior ischemic optic neuropathy

(PION), 85

Postpartum angiopathy, 251

PR3-ANCA, 12, 21, 22, 33, 37, 111, 120, 142,

143, 145, 148, 151, 162, 164, 425, 428

Preterm delivery, 215, 217, 218

Pro-angiogenic molecules, 4

Propylthiouracil, 34, 44, 272

Proteinase 3 (PR3), 10, 12, 21, 32–34, 37,

43–47, 111, 120, 121, 123, 141–145,

147, 149–151, 162, 163, 267

Pseudotumor cerebri, 324, 326

Pulmonary artery aneurysm (PAA), 230, 398

Pulmonary capillaritis, 110–112, 114, 151,

348, 353

Pulmonary hypertension, 4, 56–57

Pulmonary-renal vasculitis syndrome, 113

Purpura/asthenia/arthralgia, 334



R

Radiation induced arteritis, 399

Radiculoplexus neuropathy, 272–273

Raynaud’s phenomenon, 84, 324, 335, 381

Reactive oxygen species (ROS), 45, 46, 120,

147, 150, 151

Relapsing polychondritis, 57

Remicade, 182

Retinal vasculitis, 227–229, 398

Retinitis, 282

Retroperitoneal fibrosis, 313, 399

Revascularization, 90, 372–373, 396

Reversible cerebral vasoconstriction syndrome

(RCVS), 250, 379

Rheumatoid factor (RF), 37, 38, 89, 240, 263,

270, 291, 334, 349, 350

Rheumatoid vasculitis, 24, 345, 350–356

Ribavirin, 274, 340, 410

Rituximab, 48, 60, 115, 126, 135, 159, 160,

164, 201, 211, 215, 242, 252, 255,

272–274, 286, 295, 312, 315, 319, 328,

340, 354, 355, 408–410, 426–429



S

Saddle-nose deformity, 121

Sarcoidosis, 57, 260, 261, 263, 271, 282, 285,

300, 302, 304–307

Schnitzler syndrome, 326

Selectin, 364



438

Sensorineural hearing loss, 23, 112, 177, 180,

291, 292, 295

Single nuclear polymorphism (SNP)

rs6871626, 70

Single-organ vasculitis (SOV), 23

Small vessel vasculitis (SVV), 6, 16, 18, 19,

21–23, 203, 233–243, 346, 348, 388,

390, 397

Storiform fibrosis, 317, 318

Subarachnoid hemorrhage, 250, 292

Sweet’s syndrome, 228

Sympathectomy, 373

Systemic hypertension, 56

Systemic lupus erythematosus (SLE), 23, 43,

47, 101, 151, 242, 246, 260, 261, 268,

282, 294, 300, 302, 305, 307, 327,

345–350, 369

Systemic vasculitides, 6, 7, 16, 23, 36, 37, 46,

246, 266, 271, 300, 303, 407–423



T

Takayasu’s arteritis, 6, 19, 20, 25, 36, 37,

53–60, 63–72, 79, 213, 215–216, 251,

265, 390, 407, 408, 410

T cell tubulitis, 411

Temporal artery biopsy, 82, 83, 88, 92, 103,

389, 390

Testicular arteritis, 23

Therapeutic angiogenesis, 373

Thromboangiitis obliterans, 362–374,

381, 395

Thrombomodulin, 3

Thrombotic microangiopathy, 346

TNFα-inhibitors, 59, 285–286, 412

Tocilizumab, 26, 59, 95, 115, 286, 295,

328, 414

Toll-like receptor (TLR), 45, 206, 338

Tongue claudication, 86, 94

Transforming growth factor- (TGF)-β, 58,

204, 315

Transient ischemic attack, 220, 378, 398

Transthoracic echocardiography, 180, 184



Index

Trimethoprim–sulfamethoxazole, 428

Tumor necrosis factor (TNF), 45, 215,

412, 413

Twenty (OH)-vitamin D, 184

Type IV collagen, 198, 206

Tyrosine phosphatase-1, 290



U

Ulcerative colitis, 34, 64–72, 101

Ulcerative keratitis, 353

Urticarial vasculitis (UV), 237, 241, 242,

322–328, 336, 410, 412, 415

Ustekinumab, 71, 72

Uveitis, 23, 122, 227, 229, 281–287, 302, 305,

324, 326, 327, 398, 413



V

Valacyclovir, 272

Variable vessel vasculitis (VVV),

18, 23, 289

Vascular cell adhesion molecule-1 (VCAM-1),

6, 148, 149, 364

Vascular endothelial growth factor (VEGF),

58, 148, 204, 373

Vasculitis mimics, 400

Vena cava thrombosis, 220, 230

Vestibular dysfunction, 23, 121

Vogt-Koyanagi-Harada

syndrome, 294

von Willebrand factor (vWF), 4, 46



W

Wa (VH1-69) cross-reactive idiotype, 336

Wegener’s granulomatosis (WG), 6, 10,

20–21, 25, 43, 114, 119, 120, 130,

134, 408



X

X-ray coronary angiography, 180



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5 Treating Relapses, Refractory Disease and Other Agents

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