原発性胆汁性胆管炎（PBC）診療ガイドライ …

1 PBC 2017 2017 3 1 (2011 ) Primary biliary cirrhosis: PBC 1851 Addison & Gull 1) 1950 Ahrens 2) Primary biliary cirrhosis chronic non-suppurative destructive cholangitis:CNSDC Anti-mitochondrial antibody: AMA PBC AMA ursodeoxycholic acid.

2 UDCA 1980 PBC UDCA UDCA PBC PBC 2009 PBC AASLD, EASL PBC 2011 3 COI PBC WG WG 2 2017 2016 Primary biliary cholangitis.

3 PBC 1950 (Primary biliary cirrHosis: PBC) 2011 A) (CQ) B) CQ 2011 UDCA CQ CQ 2011 GRADE AIH 2014 CQ CQ GRADE UDCA PBC FDA obetecholic acid PBC PBC 2017 3 COI ( ), ( )

4 WG , , , 3 .. 8 I .. 8 .. 8 .. 9 .. 10 .. 11 .. 15 .. 15 .. 15 II .. 18 .. 18 .. 18 .. 18 .. 18 .. 20 .. 21 .. 21 .. 22 PBC .. 23 PBC .. 24 PBC .. 25 I .. 25 QI-1: (PBC) .. 25 QI-2: .. 25 QI-3: .. 25 QI-4: .. 26 QI-5: .. 26 QI-6: .. 26 QI-7 PBC .. 26 QI-8.

5 27 QI-9: .. 27 II PBC .. 28 QII-1:PBC 2017 .. 28 QII-2:PBC .. 28 4 III PBC .. 29 UDCA .. 29 QIII-1-1: UDCA .. 29 QIII-1-2: UDCA .. 30 QIII-1-3: UDCA .. 30 QIII-1-4: UDCA .. 30 QIII-1-5 UDCA 2017 .. 31 QIII-1-6: UDCA 2017 .. 31 Bezafibrate .. 33 QIII-2-1: Bezafibrate 2017 .. 33 QIII-2-2: Bezafibrate .. 33 .. 33 QIII-3-1 .. 33 QIII-3-2 .. 33 QIII-3-3 .. 34 IV .. 35 .. 35 QIV-1-1: Cholestyramine PBC .. 35 QIV-1-2: PBC .. 35 QIV-1-3: Rifampicin PBC .. 35 QIV-1-4: Narlfurafin PBC 2017.

6 35 .. 36 QIV-2-1: .. 36 QIV-2-2: Bisfhosphonate PBC .. 36 QIV-2-3 vitaminD3 vitaminK2 PBC .. 36 .. 37 QIV-3-1: cevimeline pilocarpine PBC .. 37 V PBC .. 38 .. 38 QV-1-1: .. 38 QV-1-2: .. 38 QV-1-3: .. 39 QV-1-4 PBC .. 39 QV-1-5: .. 39 QV-1-6: .. 40 5 .. 41 QV-2-1: .. 41 QV-2-2: .. 41 VI .. 42 .. 42 QVI-1-1: .. 42 QVI-1-2: .. 44 QVI-1-3: PBC .. 44 QVI-1-4: .. 44 .. 45 QVI-2-1.

7 45 QVI-2-2: .. 45 QVI-2-3: PBC .. 45 QVI-2-4: PBC .. 45 6 (2011 ) PBC PBC PubMed-MEDLINE Clinical Trial, RCT, Meta-Analysis 01/1998 12/2009 Primary biliary cirrhosis English UDCA 1b UDCA 1987 Poupon Lancet UDCA PBC PBC 2009 PBC AASLD, EASL Inpact Facotr.

8 Minds( ) 1a 1b 2a 2b 3 4 5 6 A B C1 C2 D 7 2017 CQ PBC (2011 ) PubMed-Medline Cochrane Library CQ WG GRADE Minds ( ) (2014 ) A B C D.

9 ( ) 8 I 27 primary biliary cholangitis PBC (symptomatic)PBC(sPBC) asymptomatic PBC (aPBC) sPBC aPBC sPBC mg/dl s2 PBC s1 PBC 1. ALP GTP antimitochondrial antibodies AMA 90% IgM 2. chronic non-suppurative destructive cholangitis CNSDC 3.

10 4. PBC 1 CNSDC PBC 2 AMA CNSDC PBC compatible 3 AMA PBC 9 PBC 27 A 1 1) CNSDC PBC 2) AMA CNSDC PBC compatible 2 3) AMA PBC 2 CT A 2 PBC 1 IgG4 2 3) ALP, -GTP MEMO ELISA AMA PBC 90% ELISA AMA M2 PBC M2 70kDa, 50kDa, 47kDa, 40kDa 70kDa M2 PDC.