Case Report
Copyright ©The Author(s) 2019.
World J Gastroenterol. Sep 21, 2019; 25(35): 5388-5402
Published online Sep 21, 2019. doi: 10.3748/wjg.v25.i35.5388
Table 1 Prevalence and incidence of autoimmune hepatitis among different countries in the world1
CountryPrevalenceIncidenceStudy period
Asia-Pacific
Taiwan[7]NA0.52000 -2004
India[8]1.5NA1999 -2002
Singapore[9]4.0NA1990 -1996
South Korea[10]4.81.12009 -2013
Brunei Darussalam[11]5.6NANA
Australia[12]8.0NANA
Israel[13]11.00.71995 -2010
Japan[14]23.42.22004 -2014
New Zealand[15]24.52.02001 -2008
Europe and America
Spain[6]NA1.12003
Finland[3]14.31.11995 -2015
Norway[16]16.91.91986 -1995
Sweden[2]17.31.21989 -2011
Netherlands[5]18.31.11967 -2011
Denmark[4]23.91.71994 -2012
United States (Alaska)[17]42.9NA1984 -2000
Table 2 Baseline characteristics of 13 human immunodeficiency virus-infected patients at the time of autoimmune hepatitis diagnosis
CaseSexAgeHIV status
Presentation, duration (mo)Lab values1
AIH score
Duration (yr)Current ART regimen, duration (year or month)Viral load, duration (year or month)CD4+ count in 2 and 1 years ago and at AIH presentationASTALTALPTBAlbuminGlobulinANA titerASMAIgG
1M477TDF/FTC/EFV, 4 yr< 40, 3 yr761, 885, 963Rising ALT, 10 mo1212631450.84.44.81:80+29307
2M4421TDF/FTC/EFV, 1 yr< 40, 5 yr588, 669, 798Rising ALT, 2 mo1301721720.44.541:80-13105
3M6110TDF/FTC/EFV, 8 yr< 40, 6 yr496, -, 646Rising ALT, 31 mo1681771890.84.04.8> 1280-18906
4M261TDF/3TC/EFV, 6 mo< 40, 1 mo-, -, 82Rising ALT, 7 mo1991971060.84.64.6< 1:40-19205
5F307TDF/3TC/EFV, 6 yr< 40, --, -, 772Rising ALT, 9 mo2361571910.43.65.9> 1:1280-41007
6F296TDF/FTC/EFV, 6 yr< 40, --, -, 731Rising ALT, 10 mo1081331251.04.13.71:320-17105
7F5725TDF/3TC/EFV, 8 yr< 40, 7 yr583, 477, 384Rising ALT, 24 mo106493570.63.76.51:2560-23337
8F4010TDF/3TC/EFV, 6 yr< 40, 5 yr-, 415, 480Rising ALT, 4 mo2693532370.74.13.9< 1:40+18605
9F466TDF/FTC/EFV, 3 yr< 40, --, -, 451Rising ALT, 20 mo1782302621.43.84.2> 1:1280+20507
10F342TDF/FTC/EFV, 1 yr< 40, 10 mo-, 377, 557Rising ALT, 11 mo1912051121.33.84.11:320+25807
11F312TDF/FTC/EFV, 6 mo< 40, 1 mo-, -, 563Jaundice, 1 mo3932661336.33.15.11:320+37107
12F4013TDF/FTC/EFV, 3 yr< 40, 3 yr320, 298, 216Rising ALT, 6 mo3141503042.03.36.8> 1:2560-50507
13M394ABC/RPV/3TC, 1 yr< 40, 2 yr-, 52, 310Rising ALT, 23 mo631241000.74.14.41:80-22507
Table 3 Treatment and outcome of autoimmune hepatitis in human immunodeficiency virus-infected patients
Induction regimen
Time to ALT normalization (mo)Maintenance regimen
OutcomeFollow up duration (mo)Last CD4 countLast HIV VL (copies/mL)
Prednisolone (mg/d)Azathioprine (mg/d)Prednisolone (mg/d)Azathioprine (mg/d)
13009.5550Minor flare43.4775< 40
22007.615100No relapse12.8799< 40
33008.2050No relapse35.4451< 40
41---------
54001.02.50No relapse10.3772< 40
63002.610100No relapse5.41841< 40
715250.90MMF 1000 mg/dayNo relapse30.2444< 40
830502.3050No relapse65.8513< 40
9*---------
102001.4050No relapse22.6632< 40
113007.6050No relapse24.9630< 40
122003.0575No relapse19.7176< 40
13300Remain in the induction phase-4.9--
Table 4 Baseline characteristics at autoimmune hepatitis presentation of previously reported human immunodeficiency virus cases
No.Sex, age (yr)HIV status
PresentationANA titerASMA titerTreatmentOutcome
HIV DurationART durationViral loadCD4
1[21]NANANA< 481011NA1:6401:40P+ ZRemission
2[21]NANANA< 48456NANeg1:20P+ ZRemission
3[21]NANANA< 48783NA1:401:320P+ ZRemission
4[21]NANANA< 48688Acute liver failure1:6401:160LTRelapse at 4 yr after LT
5[21]NANANA< 48653n/a1:12801:20P+ ZRemission
6[22]M, 54NA3 yr5104357 ALT*1:401:640P+ ZRemission
7[22]F, 55NANA< 50174 ALTNeg1:40P+ ZRelapse
8[22]F, 49NANA69318286 ALTNegNegP+ ZDied
9[22]F, 56NANA232734331 ALT1:401:20PDied
10[23]M, 21Newly diagnosed07900753JaundiceNegNegPRelapse
11[23]F, 59n/a7 mo-504 ALTPosPosP+ ZRemission
12[24]M, 263 yr1 yr-NAJaundice1:160NegP+ ZRelapse
13[25]M, 408 yr8 yrND832 ALTNeg1:60P+ ZRelapse
14[25]F, 4420 yr7 yrND823Acute hepatitis*1:6401:180PRemission
15[26]M, 527 yr7ND641 ALT1:2560NAPRemission
16[27]F, 449 mo9 moND526 ALT1:1601:160P+ ZMinor flare during taper steroid
17[28]M, 29Newly diagnosed07122259 ALTNegNegnoneResolved spontaneously
18[28]F, 452 yr7 moND297 ALT1:640NegP+ZRemission
19[28]F, 6510 yrn/aND922 ALT1:1601:320P+Z → MMFFlare
20[29]M, 382 yr after AIH diagnosed081000216 ALT1:3201:40ARTALT improved after ART initiaion
21[30]F, -15 yr12 yrND634Acute hepatitis1:20000NegPRemission
22[31]F, 4820 yrNAND> 250Acute hepatitis1:801:300PDied
23[32]F, 23NA8 moND523 Jaundice and ALT*PosNegP ± ZRemission
24[32]F, 31NA7 mo< 40277PosNeg
25[32]F, 42NA10 mo-593PosPos
26[32]F, 45NA6 mo< 40253NegPos
27[32]F, 35NA41 moND339NegPos
28[32]M, 39NA77 moND416NegPos
29[32]F, 39NA4 moND357NegPos
30[32]F, 43NA4 moND876PosNeg
31[32]F, 36NA17 moND693NegPos
32[33]F, 4220 yrNAND232Acute hepatitis1:1280NegPRemission
33[34]F, 51NA11 mo94417Acute liver failure1:2561:80PDied
34[35]F, 3813 yrNA6392436NA1:400PosBRemission
35[36]M, 382 yr08072762Acute hepatitis1:6401:160P+ZRemission
36[37]F, 7032 yr24 yr< 50938Decompensated cirrhosis*1:640NAPRemission
37[38]M, 4313 yr2 yrND900↑ ALT1:160NAPRemission
38[39]F, 434 yr18 moND425Acute hepatitis1:8000NegP+ZFlare during tapering steroid
Table 5 Demographics of non-human immunodeficiency virus infected patients with autoimmune hepatitis
CountrynFemale (%)Age at onsetCirrhosis1Disease onsetANA+ASMA+IgG > ULN
Asia-Pacific
South Korea[10]40858660-6932.3%----
Japan[46]16828760-696.7% (P)11.7% acute hepatitis 79.6% chronic hepatitis90.7%40.9%n/a
South Korea[47]343--23%46.4% acute onset 30.6% asymptomatic94.2%23.0%78.2%
New Zealand[15]1387150-5936% (P, R)-56%57%97%
Israel[13]100954822.3% (P)35% asymptomatic91%55%n/a
Taiwan[7]48775835.4% (P)12.5% asymptomatic 41.7% acute onset 45.8% insidious onset97.8%18.2%72.9%
Japan[14]48--8.3% (C+R)68.8% acute onset 31.2% chronic onset100%22.9%79.2%
Australia[12]42745324% (P)-n/a65.8%n/a
India[8]38893034.2% (C±P)13.1% acute hepatitis 39.4% acute onset 50% chronic onset39.4%63.1%n/a
Singapore[9]24926342% (C)2/3 insidious onset79.2%20.8%n/a
Brunei Darussalam[11]19795221.1% (P)53% asymptomatic100%47.4%n/a
Europe and America
Denmark[4]1721727028.3% (P)----
Netherlands[5]131378Middle age12% (P)-49%58%83%
Finland[3]8877655-64-----
Sweden[2]6347350-6028.1%----
Alaska[48]71----79.1%60%66%
United States (Alaska)[17]4992520%34.7% acute hepatitis 65.3% asymptomatic---
Norway[16]25806828% (P)-68%40%n/a
Spain[6]199545-54-----
Table 6 Simplified diagnostic scoring system of autoimmune hepatitis
ParametersScore
I Seroimmunological test (maximum 2 points)
ANA or SMA +≥ 1:40+1
ANA or SMA +≥ 1:80+2
LKM +≥ 1:40+2
SLA +Any titer+2
II IgG or immunoglobulin level> ULN+1
> 1.1 × ULN+2
III Histological findingCompatible+1
Typical+2
IV Absence of viral hepatitisNo0
Yes+2