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, 114 115 classification of, 2 comorbidities of management of, 11 12 EGPA, 12 14. 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, 114 118 vasculitis in APS and, 110 111 Antiphospholipid syndrome (APS) mimicking primary systemic vasculitis, 147 148 vasculitis in, 109 123 antiphospholipid antibodies and, 110 111, 114 118. See also specific types case reports of, 113 114 future considerations in, 118 introduction, 109 110 manifestations of, 111 114 Apheresis in cryoglobulinemia management, 102 APS. See Antiphospholipid syndrome (APS) Rheum Dis Clin N Am 41 (2015) 161 166 http://dx.doi.org/10.1016/s0889-857x(14)00116-1 rheumatic.theclinics.com 0889-857X/15/$ see front matter ª 2015 Elsevier Inc. All rights reserved.
162 B Behçet syndrome (BS), 80 85 APS and, 117 118 described, 80 diagnosis of, 81 82 epidemiology of, 80 management of, 83 85 gastrointestinal disease, 85 joint disease, 84 mucocutaneous disease, 83 84 neurologic disease, 85 ocular disease, 84 vascular disease, 84 85 pathogenesis of, 81 symptoms of, 81 Brouet classification of cryoglobulinemia, 94 BS. See Behçet syndrome (BS) C Calciphylaxis mimicking primary systemic vasculitis, 144 146 Central nervous system (CNS) primary angiitis of, 47 62. See also Primary angiitis of central nervous system (PACNS) CNS. See Central nervous system (CNS) Cogan syndrome (CS), 75 80 described, 75 diagnosis of, 78 79 epidemiology of, 75 76 management of, 79 80 pathogenesis of, 76 symptoms of, 76 78 Computed tomography angiography (CTA) Cryoglobulinemia, 93 108 Brouet classification of, 94 clinical features of, 95 97 diagnosis of, 98 99 epidemiology of, 94 introduction, 93 94 laboratory tests for, 97 pathogenesis of, 94 95 pathology of, 97 98 treatment of, 99 102 apheresis in, 102 for infectious mixed CryoVas: HCV related, 100 101 for infectious mixed CryoVas: non HCV related, 101 102 for mixed CryoVas, 100 for type I cryoglobulinemia, 99 100
163 CS. See Cogan syndrome (CS) Cutaneous lesions APS and, 111 112 D DAH. See Diffuse alveolar hemorrhage (DAH) Diffuse alveolar hemorrhage (DAH) APS and, 112 113 E EEG. See Electroencephalography (EEG) EGPA. See Eosinophilic granulomatosis with polyangiitis (EGPA) Electroencephalography (EEG) Eosinophilic granulomatosis with polyangiitis (EGPA), 12 14 ANCA with, 13 clinical features of, 12 13 tissue biopsy in, 13 treatment of, 13 14 F Fibromuscular dysplasia (FMD) mimicking primary systemic vasculitis, 142 144 FMD. See Fibromuscular dysplasia (FMD) G GCA. See Giant-cell arteritis (GCA) Giant-cell arteritis (GCA) APS and, 116 117 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, 153 155 K Kawasaki disease (KD), 63 73 clinical features of, 66 68 controversies related to, 71
164 Kawasaki (continued) differential diagnosis of, 69 epidemiology of, 64 65 genetic factors in, 66 introduction, 63 64 laboratory findings in, 68 management of, 69 70 pathophysiology of, 65 66 prognosis of, 70 71 risk factors for, 65 KD. See Kawasaki disease (KD) L Large vessel vasculitis (LVV), 125 140 APS and, 116 117 diagnosis of imaging in, 129 134 TAB in, 126 129 discussion, 134 135 introduction, 125 126 Livedoid vasculopathy mimicking primary systemic vasculitis, 152 153 LMPG. See Lymphomatoid granulomatosis (LMPG) LVV. See Large vessel vasculitis (LVV) Lymphomatoid granulomatosis (LMPG) mimicking primary systemic vasculitis, 149 150 M Magnetic resonance angiography (MRA) Magnetic resonance imaging (MRI) Malignant atrophic papulosis (MAP) mimicking primary systemic vasculitis, 150 151 MAP. See Malignant atrophic papulosis (MAP) P PACNS. See Primary angiitis of central nervous system (PACNS) PAN. See Polyarteritis nodosa (PAN) Polyarteritis nodosa (PAN), 33 46 classification of, 34 35 clinical features of, 37 38 cutaneous, 39 differential diagnosis of, 41 42 epidemiology of, 35 37 future considerations for, 43 histopathology of, 39 introduction, 33 laboratory features of, 38
165 pathogenesis of, 37 prognosis of, 43 radiographic features of, 39 risk factors for, 35 37 single-organ disease, 39 40 subsets of, 39 41 treatment of, 42 43 viral-associated, 40 41 Primary angiitis of central nervous system (PACNS), 47 62 in adults, 51 management of, 57 58 childhood, 51 management of, 58 clinical features of, 49 51 diagnosis of, 52 54 criteria for, 54 EEG in, 53 laboratory studies in, 52 53 neuroimaging in, 53 54 differential diagnosis of, 54 57 epidemiology of, 48 future considerations in, 59 histopathology of, 48 49 introduction, 47 48 management of, 57 59 monitoring disease activity in, 58 59 pathology of, 54 pathophysiology of, 48 prognosis of, 59 subtypes of, 51 52 Primary systemic vasculitis mimickers of, 141 160. See also specific disorders APS, 147 148 calciphylaxis, 144 146 FMD, 142 144 HES, 148 IgG4 related disease, 153 155 introduction, 141 142 livedoid vasculopathy, 152 153 LMPG, 149 150 MAP, 150 151 RCVS, 151 152 SAM, 146 147 R RCVS. See Reversible cerebral vasoconstriction syndrome (RCVS) Retinal vasculitis APS and, 112 Reversible cerebral vasoconstriction syndrome (RCVS) mimicking primary systemic vasculitis, 151 152
166 S SAM. See Segmental arterial mediolysis (SAM) Segmental arterial mediolysis (SAM) mimicking primary systemic vasculitis, 146 147 Skin small vessel vasculitis of, 21 32. See also Small vessel vasculitis of skin Small vessel vasculitis antiphospholipid antibodies and APS in, 114 115 Small vessel vasculitis of skin, 21 32 causes of, 22 23 clinical features of, 23 25 differential diagnosis of, 26 28 epidemiology of, 22 initial workup for, 25 26 introduction, 21 22 management of, 28 29 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, 126 129 V Variable vessel vasculitis APS and, 117 118 Vasculitis ANCA associated, 1 19. See also Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis in APS, 109 123. See also Antiphospholipid syndrome (APS), vasculitis in large vessel, 125 140. See also Large vessel vasculitis (LVV) primary systemic mimickers of, 141 160. See also Primary systemic vasculitis, mimickers of retinal APS and, 112 small vessel antiphospholipid antibodies and APS in, 114 115 of skin, 21 32. See also Small vessel vasculitis of skin systemic antiphospholipid antibodies and APS in, 114 118