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1 Note: Page numbers of article titles are in boldface type. A ANCA vasculitis. See Antineutrophil cytoplasmic antibody associated (ANCA) vasculitis Angiography 54 Antineutrophil cytoplasmic antibody correlation with disease activity, 4 detection of, 2 3 in EGPA, 13 measurements of, 4 specificity of implications of, 3 4 Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, 1 19 APS and, classification of, 2 comorbidities of management of, EGPA, See also Eosinophilic granulomatosis with polyangiitis (EGPA) future considerations for, 12 introduction, 1 2 terminology related to, 2 tissue biopsy in, 4 5 treatment of, 5 11 historical perspective, 5 induction therapy in, 8 9 maintenance therapy in, 9 11 principles of, 5 7 Antiphospholipid antibodies APS and in systemic vasculitis, vasculitis in APS and, Antiphospholipid syndrome (APS) mimicking primary systemic vasculitis, vasculitis in, antiphospholipid antibodies and, , See also specific types case reports of, future considerations in, 118 introduction, manifestations of, Apheresis in cryoglobulinemia management, 102 APS. See Antiphospholipid syndrome (APS) Rheum Dis Clin N Am 41 (2015) rheumatic.theclinics.com X/15/$ see front matter ª 2015 Elsevier Inc. All rights reserved.

2 162 B Behçet syndrome (BS), APS and, described, 80 diagnosis of, epidemiology of, 80 management of, gastrointestinal disease, 85 joint disease, 84 mucocutaneous disease, neurologic disease, 85 ocular disease, 84 vascular disease, pathogenesis of, 81 symptoms of, 81 Brouet classification of cryoglobulinemia, 94 BS. See Behçet syndrome (BS) C Calciphylaxis mimicking primary systemic vasculitis, Central nervous system (CNS) primary angiitis of, See also Primary angiitis of central nervous system (PACNS) CNS. See Central nervous system (CNS) Cogan syndrome (CS), described, 75 diagnosis of, epidemiology of, management of, pathogenesis of, 76 symptoms of, Computed tomography angiography (CTA) Cryoglobulinemia, Brouet classification of, 94 clinical features of, diagnosis of, epidemiology of, 94 introduction, laboratory tests for, 97 pathogenesis of, pathology of, treatment of, apheresis in, 102 for infectious mixed CryoVas: HCV related, for infectious mixed CryoVas: non HCV related, for mixed CryoVas, 100 for type I cryoglobulinemia,

3 163 CS. See Cogan syndrome (CS) Cutaneous lesions APS and, D DAH. See Diffuse alveolar hemorrhage (DAH) Diffuse alveolar hemorrhage (DAH) APS and, E EEG. See Electroencephalography (EEG) EGPA. See Eosinophilic granulomatosis with polyangiitis (EGPA) Electroencephalography (EEG) Eosinophilic granulomatosis with polyangiitis (EGPA), ANCA with, 13 clinical features of, tissue biopsy in, 13 treatment of, F Fibromuscular dysplasia (FMD) mimicking primary systemic vasculitis, FMD. See Fibromuscular dysplasia (FMD) G GCA. See Giant-cell arteritis (GCA) Giant-cell arteritis (GCA) APS and, H Henoch-Schönlein purpura (HSP) APS and, 115 HES. See Hypereosinophilic syndrome (HES) HSP. See Henoch-Schönlein purpura (HSP) Hypereosinophilic syndrome (HES) mimicking primary systemic vasculitis, 148 I IgG4 related disease. See Immunoglobulin G4 (IgG4) related disease Immunoglobulin G4 (IgG4) related disease mimicking primary systemic vasculitis, K Kawasaki disease (KD), clinical features of, controversies related to, 71

4 164 Kawasaki (continued) differential diagnosis of, 69 epidemiology of, genetic factors in, 66 introduction, laboratory findings in, 68 management of, pathophysiology of, prognosis of, risk factors for, 65 KD. See Kawasaki disease (KD) L Large vessel vasculitis (LVV), APS and, diagnosis of imaging in, TAB in, discussion, introduction, Livedoid vasculopathy mimicking primary systemic vasculitis, LMPG. See Lymphomatoid granulomatosis (LMPG) LVV. See Large vessel vasculitis (LVV) Lymphomatoid granulomatosis (LMPG) mimicking primary systemic vasculitis, M Magnetic resonance angiography (MRA) Magnetic resonance imaging (MRI) Malignant atrophic papulosis (MAP) mimicking primary systemic vasculitis, MAP. See Malignant atrophic papulosis (MAP) P PACNS. See Primary angiitis of central nervous system (PACNS) PAN. See Polyarteritis nodosa (PAN) Polyarteritis nodosa (PAN), classification of, clinical features of, cutaneous, 39 differential diagnosis of, epidemiology of, future considerations for, 43 histopathology of, 39 introduction, 33 laboratory features of, 38

5 165 pathogenesis of, 37 prognosis of, 43 radiographic features of, 39 risk factors for, single-organ disease, subsets of, treatment of, viral-associated, Primary angiitis of central nervous system (PACNS), in adults, 51 management of, childhood, 51 management of, 58 clinical features of, diagnosis of, criteria for, 54 EEG in, 53 laboratory studies in, neuroimaging in, differential diagnosis of, epidemiology of, 48 future considerations in, 59 histopathology of, introduction, management of, monitoring disease activity in, pathology of, 54 pathophysiology of, 48 prognosis of, 59 subtypes of, Primary systemic vasculitis mimickers of, See also specific disorders APS, calciphylaxis, FMD, HES, 148 IgG4 related disease, introduction, livedoid vasculopathy, LMPG, MAP, RCVS, SAM, R RCVS. See Reversible cerebral vasoconstriction syndrome (RCVS) Retinal vasculitis APS and, 112 Reversible cerebral vasoconstriction syndrome (RCVS) mimicking primary systemic vasculitis,

6 166 S SAM. See Segmental arterial mediolysis (SAM) Segmental arterial mediolysis (SAM) mimicking primary systemic vasculitis, Skin small vessel vasculitis of, See also Small vessel vasculitis of skin Small vessel vasculitis antiphospholipid antibodies and APS in, Small vessel vasculitis of skin, causes of, clinical features of, differential diagnosis of, epidemiology of, 22 initial workup for, introduction, management of, pathophysiology of, 22 prognosis of, 29 T TAB. See Temporal artery biopsy (TAB) TAK. See Takayasu arteritis (TAK) Takayasu arteritis (TAK) APS and, 117 Temporal artery biopsy (TAB) in LVV diagnosis, V Variable vessel vasculitis APS and, Vasculitis ANCA associated, See also Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis in APS, See also Antiphospholipid syndrome (APS), vasculitis in large vessel, See also Large vessel vasculitis (LVV) primary systemic mimickers of, See also Primary systemic vasculitis, mimickers of retinal APS and, 112 small vessel antiphospholipid antibodies and APS in, of skin, See also Small vessel vasculitis of skin systemic antiphospholipid antibodies and APS in,

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