1
14163 1611 (2002) 32007 3 3 196 2
1416 ( ) Medsger modified Rodnan total skin thickness score EBM placebo control ACEEBM EBM 3
1611 4
5
(Systemic Sclerosis : SSc) 1980 (1) 1) 200310 (2) 2003 Scl-70 1 (4) 6
(4) 90% Subcommittee for scleroderma criteria of the american rheumatism association diagnostic and therapeutic criteria committee: Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980, 23:581-90 7
LeRoy & Medsgerdiffuse cutaneous SSc(dSSc)limited cutaneous SSc (lssc) 2 1,2) dssclssc dssc lssc 1) 1988, 30: 1499-1505 LeRoy EC, Black C, Fleischmajer F, Jablonska S, Krieg T, Medsger TA Jr et al : Scleroderma (systemic sclerosis) : classification, subsets and pathogenesis. J Rheumatol. 1988, 15:202-204 8
a. Medsger (severity) damage () activity ( ) 1) modified Rodnan total skin thickness score (TSS) 2) endpoint TSS 12 TSS score b. dssc disease modifying drugs () lssc 3) EBMdisease modifying drugs disease modifying drugs dsscstage () disease modifying drugs Medsger TA Jr, Silman AJ, Steen VD, Black CM, Akesson A et al.: A disease severity scale for systemic sclerosis: Development and testing. J Rheumatol 1999, 26: 2159-2167 Clements P, Lachenbruch P, Siebold J, White B, Weiner S et al.: Inter and intraobserver variability of total skin thickness score (Modified Rodnan TSS) in systemic sclerosis. J Rheumatol 1995, 22: 1281-1285 Seibold JR, Furst DE, Clements PJ: Why everything (or nothing) seems to work in the treatment of scleroderma. J Rheumatol 1992, 19: 673-675 9
Medsger () 10
a. 1. Medsger Medsgermodified Rodnan total skin thickness score (TSS)Medsger579 0 (Normal) 1 (Mild) 2 (Moderate) 3 (Severe) 4 (Endstage) TSS= 0 4 1-14 48 15-29 23 30-39 12 40+ 12 (Medsger TA et al. J Rheumatol 26: 2159-2167, 1999) 2. MedsgerSSc Medsger151SSc 0 (Normal) 1 (Mild) 2 (Moderate) 3 (Severe) 4 (Endstage) TSS= 0 7 1-14 64 15-29 24 30-39 4 40+ 1 MedsgerSSc1 (Mild)3 (Severe)4 (Endstage) Medsger1 (Mild)2 (Moderate)TSS15 10SSc 0 (Normal) 1 (Mild) 2 (Moderate) 3 (Severe) 4 (Endstage) TSS= 0 7 1-9 50 10-19 23 20-29 15 30+ 5 3 (Severe)4 (Endstage)1 (Mild)1 (Mild) 3. EndstageVery severe 11
0 (Normal) 1 (Mild) 2 (Moderate) 3 (Severe) 4 (Very Severe) TSS*= 0 1-9 10-19 20-29 30 + *modified Rodnan total skin thickness score (TSS) TSS b. 1. 1diffuse cutaneous SSc 2 3 2030 mg/ 20 mg/ 20 mg/30 mg/ TSS30 (RNA) 10mg/ 2. SSc Tashkin DP et al. N Engl J Med 354: 2655, 2006 12
a. %VC/%DLco1.4 1. Interstitial lung disease; ILD 0normal 1mild 2moderate 3severe 4very severe %VC 80% %VC 65-79% %VC 50-64% %VC 50% XCTX CT 2. Pulmonary hypertension; PH 0normal 1mild 2moderate 3severe 4very severe PH PHWHO PHWHO PHWHO PHWHO PHPAHPH PH XN-T probnp PH 35mmHg35-50mmHg 50mmHgPH 13
35mmHg 35mmHg PH 3PH 25mmHg>30mmHg 15mmHg 3 PH PHCT WHO 14
b. 1. Interstitial lung disease; ILD ILD ILD %VC%DLco KL-6SP-D alveolitis CTHRCTBALF HRCT BALF HRCTBALF ILD 12 D- 4 15
1 2 16
D- 1-2mg/kg/12 500-750mg/m 3 1-316 0.2-0.5mg/kg/ 50-100mg/ 125-250mg/ 1-2mg/kg/ 100-250mg/ 1-5mg/ 2. Pulmonary hypertension; PH PH21 PH 30mg/WHOI6 WHOII SpO 2 >90% WHOIII PDE5 3 WHOII 3 2 PH 17
ILD PH 18
PDE5 CR 0.5-1mg/kg/ 40-120mg/ 60-180g/ 2-10ng/kg/ 125-250mg/ 50-100mg/ 19
a 1 Barrett 24pH 2 CT 3 121517.53106 20 A. 0.Normal Normal 1.Mild Mild 2.Moderate Moderate 3.Severe 4.Very severe B 0.Normal 1.Mild 2.Moderate 3.Severe 4.Very severe GERD
Body mass Index D-XyloseSchilling b. 1 1 H2 22Moderate 2 F2 21
QOL 22
a. ANCA 0Normal 1Mild 2Moderate 3Severe 0.9 1.2 mg/dl 12 1.3 2.9 mg/dl 34 3 mg/dl 4Very severe 0Normal ACE2 1 1 110mmHg 2 23
Keith-Wegener RNA ANCA P-ANCAMPO-ANCA TTP/HUS LDH ADAMTS13ADAMTS13 b. 1 ACE25mg 12.512170150mmHg 48 ACE AT1 ACE CAPD 24
1 2 BUN 60mg/dlCr 6mg/dl 3 4 ACE 25
26
a. 6 160150130 95 7595 5075 2550 25 0 (normal) 1 (mild) 2 (moderate) 3 (severe) 0 13 47 8 b. 27
0 (Normal): Normal 1 (Mild): Raynaud s phenomenon 2 (Moderate): Digital pitting ulcers 3 (Severe): Other skin ulcerations 4 (Very severe): Digital ganegrene b. 1. 1 Ca blocker: diltiazem ()nifedipinenicardipine Beraprost sodium () Sarpogrelate hydrochloride () Limaprost alfadex () ACE inhibitor, ARB: losartan ()captopril () Tocophenol nicotinate(n) 2. 234 PGE1 PGE1agatroban 3. 23ulcer U-pasta 28