Circulation Journal Vol. 72, Suppl. IV, 2008 1319
Churg-Strauss ANCA ANCA 1 1 1320 Circulation Journal Vol. 72, Suppl. IV, 2008
ANCA 1 ANCA ANCA 2 Circulation Journal Vol. 72, Suppl. IV, 2008 1321
2 2 2 IgA ANCA MPO-ANCA PR-ANCA MPO- ANCA IC PR3- ANCA PAN MPA AGA WG IgA IC HSP ADL/QOL steroid sparing DNA DNA RF MRA 1322 Circulation Journal Vol. 72, Suppl. IV, 2008
g // -D Cox- A IFN- PSL.mg/kg/PSL.mg/kg/ PSL.mg/kg/ PSL.mg/kg/ /L PSL.mg/kg/ /L STTMP/SMX g g/g/g/g/g/ A=mg mg/mg/ /L/L YAM T SD T D RR=. Circulation Journal Vol. 72, Suppl. IV, 2008 1323
3 1 a b ACR 2 A B C 1324 Circulation Journal Vol. 72, Suppl. IV, 2008
Takayasu s arteritis pulseless disease aortitis syndrome T Numano 3 perivascular cuffing Langhans a ba+ / b+ / C+ P+ p. Circulation Journal Vol. 72, Suppl. IV, 2008 1325
33.0 20.4 2.9 2.1 0.4 1.7 4.8 5.0 5.9 31.2 46.4 24.9 11.3 12.3 19.3 20.0 14.8 1.6 7.4 41.1 7.9 16.5 22.9 4 CRP PTAPTT AT IgGIgACC HLABHLAB HLAB Digital Subtraction AngiographyDSA 1326 Circulation Journal Vol. 72, Suppl. IV, 2008
DCT FDG-PET 57 PET 2030mg/ 60mg/ MTX 615mg/ CPA 50100mg/ 300750mg/m 2 CsA 3mg/kg/ AZP 2mg/kg/ MMF1.53g/ 3mg/kg 25mg 2 Circulation Journal Vol. 72, Suppl. IV, 2008 1327
1 2 1 2 3 4 5 6 7 8 9 10 3 1 2 3 4 5 6 7 8 4 1 CRP 2 3 IgGIgAC 3 C 4 4 5 H L A HLA-B52B39 5 1 CT 2 CTMRA 3 DSACTMRA 4 DSACTMRA 5 DSACTMRA 6 7 DSA 6 1 DSACTMRA 2 1 3 4 7 1328 Circulation Journal Vol. 72, Suppl. IV, 2008
8 mg/ mg/ mg/ A HLA-B CPA MTXAZP CsA MMF TNF- /CRP. mg/dl CT / Bentall CT Bentall elephant trunk staged operation Y Circulation Journal Vol. 72, Suppl. IV, 2008 1329
stent high risk stent SPECTPET hyperperfusion syndrome infrarenal MRA CT, HLA-A-B -A-B-DR HLA-B HLA-DPB* HLA-DRB* 1330 Circulation Journal Vol. 72, Suppl. IV, 2008
1331 Circulation Journal Vol. 72, Suppl. IV, 2008 rubor 6 712 7 8 9 10 11 12 5.1 74.7 20.2 11.5 41.4 40.4 37 15 16 10 19 72 43 90
A B MRA 3D-CT 150 2 3 4 5 5 1 2 3 4 5 6 7 8 9 1 2 3 4 5 PGE - CDlipoPGE 1332 Circulation Journal Vol. 72, Suppl. IV, 2008
.km/h EPC Fontaine13 Fontaine Fontaine Fontaine.. Fontaine visceral artery... Circulation Journal Vol. 72, Suppl. IV, 2008 1333
temporal arteritis giant cell arteritis... jaw claudication CRP PMR 14 cm : MRImagnetic resonance imagingfdg-pet 150 50 2 3 4 50 /Westergren 5 5 3* * 1334 Circulation Journal Vol. 72, Suppl. IV, 2008
fluorodeoxyglucose-positron emission tomography PET PSLmg/kg mg/psl.mg/. PSL mg/ PSL mg/b PSL mg/kg/b CRP mg/ mg mg/.mg mg mg/ PSL mg/ B MTX MTX ADL QOL TNF-... PMR Circulation Journal Vol. 72, Suppl. IV, 2008 1335
polyarteritis nodosa: PAN KussmaulMaier microscopic polyangiitis: MPA Churg-Strauss MPAPAN PAN..MPA PAN B PAN PAN MPA Arkin 9 :HE, :Elastica-Masson 1336 Circulation Journal Vol. 72, Suppl. IV, 2008
Arkin PAN PAN cpan CRP BUN ANCA PAN ANCA HBs PAN BHB PAN HBs PAN PAN MRA Circulation Journal Vol. 72, Suppl. IV, 2008 1337
PAN BVASBirmingham Vasculitis Activity ScoreFFSFive Factor Score PAN 15 PAN MPA ANCA MPA Chapel Hill Consensus ConferenceCHCC PAN PAN bc b CHB bc 10.mg/ kg/mg/ 1 3826 6kg 2 3 4 definite 2 probable a2 b 6 5 10,000/L 400,000/L CRP 6 SLERA 1 4 2 3 mg/+ml/..mg/kg/ CYintravenous cyclophosphamide: IVCYCY.mg/kg/ IVCY CY mg/kg/mgmg/ ml IVCY / IVCY CY 1338 Circulation Journal Vol. 72, Suppl. IV, 2008
HB MP.500 1000mg/ 3 2 : PSL 0.5 0.8mg/kg/ PSL 0.5 1mg/kg/ HB IVCY 10 15mg/kg/1,4 6 CY 0.5 2mg/kg/ MP: methylprednisolone, PSL prednisolone, IVCY: intravenous cyclophosphamide, CY: cyclophosphamide, bc16 IVCY IVCY CY bc b C HB bc Cr < 3.4mg/dl Cr > 3.4mg/dl 60 15mg/kg/pulse 12.5mg/kg/pulse 6070 12.5mg/kg/pulse 10mg/kg/pulse 70 10mg/kg/pulse 7.5mg/kg/pulse /:http://www.nanbyou.or.jp/ sikkan/ i.htm mg/ bccy b C Circulation Journal Vol. 72, Suppl. IV, 2008 1339
PAN CMV ST / / -,-D- P. jirovecii / CMV CMV CMV CMV B mg mg/ PAN b C HB PAN, ANCA MPOMPO-ANCA ANCA 1340 Circulation Journal Vol. 72, Suppl. IV, 2008
17 1 2 3 1MPO-ANCA 2CRP 3BUN 4X 1definite a2 b 1 2 2 MPO-ANCA 2probable a3 b1 MPO-ANCA 1PN 2Wegener 3Churg-Strauss 4Goodpasture 1 1 2 2 12 3 MPO-ANCA 4 p., Wegener ELK Wegener WoudeCProteinase ANCA ANCA Sequence E LX K ELK CPR-ANCA 18 Circulation Journal Vol. 72, Suppl. IV, 2008 1341
1 1E E: 2L L: 3K K 4 138266 2 2 1ELK 2 3 3 proteinase 3PR3ANCA cytoplasmic patternc-anca 1definite 1ELK3 2 ELK 2123 1 3 ELK 11231 c-pr3 -ANCA 2probable 1ELK2 2 ELK 1123 1 3ELK1c-PR3-ANCA 1CRP 2BUN 1EL 2Churg-Strauss 1 ELKEL 2 2 ELK 3EL 4EL CTMRI 5PR3-ANCA Churg-Strauss T 1342 Circulation Journal Vol. 72, Suppl. IV, 2008
19 Churg-Strauss PN Churg- Strauss 1 1 2 3 382 6 6 2 123 3 1 2 1definite 1 1 1 23Churg-Strauss 2probable 111 23Churg-Strauss 11/ 240/ 3IgE600U/m 4MPO-ANCA 5 6 12,000g/m 23 3X 4 5 6 7 Circulation Journal Vol. 72, Suppl. IV, 2008 1343
IgA IgA 20 IgMIgG IgMIgG IgGIgG HCV Raynaud 21 1. 2. 20 20 3. 4... NSAIDs / 1344 Circulation Journal Vol. 72, Suppl. IV, 2008
IgM IgG IgA, light chain IgMRF Ig IgMRF Ig ABC SLERAPNSjögren 25 25 50 RA MRA, RA MRA RA PN EA RA IgG 22 D- Circulation Journal Vol. 72, Suppl. IV, 2008 1345
RA MRA AB 2 RAHA RAPA2,560RF960IU/ml X 2 C3C4 CH50 2C1q 1987 1A 3 1A 1 BMRA MCTD 1346 Circulation Journal Vol. 72, Suppl. IV, 2008