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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.