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自身免疫性肝病 Autoimmune Liver Diseases. 王贵强 北京大学第一医院 感染疾病科. 自身免疫性肝病包括. 自身免疫性肝炎(AIH) 原发性胆汁性肝硬化(PBC) 原发性硬化性胆管炎(PSC) 自身免疫性胆管炎(AIC) 重叠综合征 (Overlap Syndromes). 自身免疫性肝病( AILD )发病机理. 宿主易感性 免疫耐受的打破 诱发因素 靶细胞的损害和疾病. 宿主易感性. AIH , PBC 女性多见, 伴随其他自身免疫疾病 PSC 男性多见 溃疡性结肠炎( 60% ) 伴随其他自身免疫疾病 - PowerPoint PPT Presentation
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Autoimmune Liver Diseases
*WANG, GQ
AIHPBCPSCAIC (Overlap Syndromes)
*WANG, GQ
AILD
*WANG, GQ
AIHPBCPSC60%HLADR3DR4dsDNA
*WANG, GQ
*WANG, GQ
*WANG, GQ
Autoimmune Hepatitis (AIH)- (4:1)Czaja AJ & Freese DK. Diagnosis and treatment of AIH. Hepatology 2002; 36:479-497
*WANG, GQ
AIHMcFarlane IG. Pathogenesis of autoimmune hepatitis. Biomed & Pharmacother. 1999; 53:255
*WANG, GQ
Potential mechanisms of immune-mediated damage in AIH
*WANG, GQ
AIH
*WANG, GQ
AIH-Interface hepatitis
*WANG, GQ
AIH-Plasma cell infiltration
*WANG, GQ
AIH N IH/PN BN LH RLC CN PPI (%) (%) (%) (%) (%) (%)
I 21 100 57.1 95.2 71.4 33.3 95.2
II 5 100 80.0 100 100 80.0 20.0
IH/PN/ RLC LH PPI CN 2002162730
*WANG, GQ
AIHSLE
*WANG, GQ
AIH N ALT AST Glo ANA LKM1 (F/M) (U/L) (U/L) (%) SMA (%)
I 21 19/2 40.5 287.2 206.1 42.6 95.2 0 18.6 II 5 5/0 12.5 389.6 319.8 26.1 0 100 19.1
IIIIII 2002162730
*WANG, GQ
AIHANASMA ILKM-1 /SLA/LP pANCA ASGPR ILC-1
*WANG, GQ
ANAANAAIH6713SMA54AIHANA 1:320 (1:40~1:16384)74ANA 1:40ANARNADNAHepG2ANAPBCPSC
*WANG, GQ
SMA SMAAIH8733ANA54SMA1:160(1:40~1:5120)80AIH1:40SMAF-tubulindesminvimentin
*WANG, GQ
(LKM1)LKM1ANASMAAIH
LKM1LKM1P-450CYP 2D6P450 2D650Kda
*WANG, GQ
/SLA/LP/SLA/LPAIH50KdatRNP(Ser)Sec) SLA/LPAIHAIH
*WANG, GQ
pANCA pANCAAIH90pANCAAIHpANCAAIHAIH
*WANG, GQ
ILC1ILC1AIHLC140AIH2032LC1LKM1LC1HCVAIH
*WANG, GQ
(-actin)AIHSMAAIHHLA DR3SMASMAAIH
*WANG, GQ
ASGPRASGPRANASMALKM1ASGPR
*WANG, GQ
AIH
ALTIgG1.5 ANASMALKM1180, 120AMA
*WANG, GQ
AIH1- AECMV EBV