Autoimmune hepatitis
1. Á¤ÀÇ : hepatocellular necrosis and inflammation with fibrosis°¡ Áö¼ÓµÇ¾î °á±¹ °£°æº¯°ú
°£ºÎÀüÀ¸·Î ÁøÇàµÇ´Â ¸¸¼ºÁúȯ.
½ÉÇÑ °æ¿ì 6°³¿ù »ç¸Á·üÀÌ 40%³ª µÈ´Ù.
¸ðµç °æ¿ì¿¡¼ autoantibody³ª ÀüÇüÀûÀÎ autoimmunityÀÇ Æ¯Â¡À» º¸ÀÌ´Â °ÍÀº ¾Æ´Ï¹Ç·Î
ÀÌ·± ÇüÅÂÀÇ ¸¸¼º°£¿°¿¡ ´ëÇØ¼ º¸´Ù Àû´çÇÑ Ç¥ÇöÀº "idiopathic" or cryptogenic
chronic hepatitisÀÏ °ÍÀÌ´Ù.
2. Immunopathogenesis
°£¼¼Æ÷¿¡ ´ëÇØ ¼¼Æ÷ ¸Å°³¼º ¸é¿ªÀÇ °ø°ÝÀ¸·Î »ý±ä´Ù.
¼±ÃµÀûÀ¸·Î ÀÚ°¡¸é¿ªÀÇ ¼ºÇâÀÌ ÀÖ´ø »ç¶÷ÀÌ È¯°æÀû ¿ä¼Ò(chemical or viral factor)¿¡ ÀÇÇØ
ƯÀÌÀû °£¼Õ»óÀÌ À¯¹ßµÈ´Ù.
* Autoimmune hepatitis¿¡ autoimmune pathogenesis°¡ °ü¿©ÇÑ´Ù´Â Áõ°Å
i) °£¿¡¼ Á¶Á÷º´¸®¼Ò°ßÀº ÁÖ·Î cytotoxic T cell°ú plasma cell·Î ±¸¼ºµÇ¾î ÀÖ´Ù.
ii) circulating autoAb(ANA, anti-smooth m Ab, thyroid), RF, hyperglobulinemia°¡ ÈçÇÏ´Ù.
iii) ´Ù¸¥ ÀÚ°¡¸é¿ªÁúȯÀÌ È¯ÀÚ¿Í Ä£Àû¿¡¼ ÈçÇÏ´Ù.
: thyroiditis, RA, autoimmune hemolytic anemia, UC, proliferative GN, juvenile DM,
Sjogren's syndrome
iv) HLA¿Í °ü·ÃµÊ : HLA B1, 8, DR3, DR4
v) glucocorticoid/immunosuppressive tx¿¡ ¹ÝÀÀÇÔ.
(1) Cellular immune Mx : pathogenesis¿¡ Áß¿äÇÔ.
in vitro study¿¡¼ lymphocyte°¡ °£¼¼Æ÷¸¦ ÆÄ±«Çϸç cytotoxic lymphocyte¿¡ ÀÇÇÑ
¸é¿ªÁ¶ÀýÀÌ °üÂûµÊ.
(2) circulating autoantibody
: ANA(homogenous pattern), smooth m(anti SM Ab), anti-LKM, Ab to "soluble liver
Ag
ÀÌ·± Ç¥ÁöÀÚµéÀÌ Áø´Ü¿¡´Â µµ¿òÀÌ µÇÁö¸¸ º´Àο¡ °ü¿©ÇÔÀº È®¸³µÇ¾î ÀÖÁö ¾Ê´Ù.
(3) Humoral immune Mx : extrahepatic manifestation¿¡ °ü¿©
arthralgia, arthritis, cutaneous vasculitis, GN
3. ÀÓ»óÀû Ư¡
chronic viral hepatitis¿Í À¯»ç
young to middle-aged women with marked hyperglobulinemia
high-titer circulating ANA
positive LE preparation(= "Lupoid" hepatitis)
°æ°ú´Â ´Ù¾çÇÏ´Ù.
mild disease or limited histologic lesion(piecemeal necrosis without bridging)
: cirrhosis´Â µå¹°´Ù.
severe symptomatic autoimmune hepatitis(20%Â÷Áö)
: aminotransferase>10¹è, marked hyperglobulinemia
aggressive histologic lesion(bridging necrosis or multilobular collapse, cirrhosis)
=> 6mo mortality°¡ 40%
* Poor prognostic signs
i) multilobular collapse at initial presentation
ii) 2ÁÖ Ä¡·áÈÄ¿¡µµ bilirubinÀÇ È£ÀüÀÌ ¾øÀ» ¶§
4. Lab
AST/ALT : Áõ°¡µÇ°í fluctuation, 100-1000U
Hypergammaglobulinemia(>2.5g/dL)
RF : common
circulating autoAb
ANA : homogenous
smooth m. Ab : less specific(chronic viral hepatitis¿¡¼µµ ÈçÇÔ)
¡ÚautoAb¿¡ µû¸¥ typeºÐ·ù
(1) Type I : young women, marked hyperglobulinemia, lupoid feature, circulating ANA
(2) Type II: children, ÁöÁßÇØ¿¡ ÈçÇÔ.
ANA(-), anti-LKM(+)
anti-LKM1(P450 IID6) : chronic hepatitis C¿¡¼µµ ¹ß°ß
anti-LKM2 : drug-induced hepatitis
anti-LKM3 : chronic hepatitis D
- 2 specific groups
type IIa(typical autoimmune) - young women, hyperglobulinemia, anti-LMK1 titer¡è
glucocorticoid tx¿¡ ¹ÝÀÀ, ¼À¯·´, ¿µ±¹¿¡¼ ÈçÇÔ.
type IIb(HCV-assocated) - HCV infection°ú °ü·Ã, older men
nl globulin, anti-LKM1 titer¡é
Interferon¿¡ ¹ÝÀÀ, ÁöÁßÇØ¿¡ ÈçÇÔ.
(3) Type III: ANA(-), anti-LKM1(-)
Ab to soluble liver antigen
5. Ä¡·á
Glucocorticoid(Pd or prednisolone)
response: 80% ±×·¯³ª cirrhosis·ÎÀÇ ÁøÇàÀº ¸·Áö ¸øÇÑ´Ù.
i) 60mg/d ¡¿ 1°³¿ù -> 20mg/d·Î À¯Áö
ii) 30mg/d + Azathioprine 50mg/d ¡¿ 1 °³¿ù -> tapering(Pd 10mg/d)
=> 18°³¿ù º´ÇÕ Ä¡·á½Ã steroidÀÇ serious life-threatening cxÀÌ 66%¿¡¼ 20%·Î °¨¼ÒÇÔ.
Azathioprine´Üµ¶ ȤÀº alternate glucocorticoid Tx´Â remission¿¡ È¿°ú¾ø´Ù.
Áõ»óÈ£Àü : ¼öÀÏ-¼öÁÖ
lab È£Àü : ¼öÁÖ-¼ö°³¿ù
Á¶Á÷ÇÐÀû È£Àü: 6-24°³¿ùµ¿¾È delayµÊ
°Ë»ç½Ç ¼Ò°ßÀÇ È£ÀüÀº Bil, globulin levelÀÌ °¨¼ÒÇϸç AlbÀÌ »ó½ÂÇÑ´Ù.
AST/ALT´Â Áï½Ã ¶³¾îÁöÁö¸¸ ÀÌ°Í ´Üµ¶ÀÇ È£ÀüÀÌ È¸º¹ÀÇ ÁöÇ¥°¡ µÉ ¼ö´Â ¾ø´Ù.
Ä¡·á±â°£ : 12-18°³¿ù
Ä¡·áÁß´ÜÈÄ Àç¹ß : 50%
PdÁß´ÜÈÄ azathioprine´Üµ¶À» Áö¼ÓÇϸé Àç¹ßÀ²À» °¨¼Ò½ÃŲ´Ù.
liver TPxÈÄ Àç¹ßÀº µå¹°´Ù.