Retrospective Study
Copyright ©The Author(s) 2021.
World J Hepatol. Dec 27, 2021; 13(12): 2113-2127
Published online Dec 27, 2021. doi: 10.4254/wjh.v13.i12.2113
Table 1 Laboratory and histological features at diagnosis of 75 children with autoimmune liver disease

All patients, n = 75
AIH, n = 54
ASC, n = 21
P value
Biochemical profile
AST U/L (NV ≤ 45)438 (129-982)678 (204-1200)150 (94-333)< 0.001
GGT U/L (NV ≤ 50)116 (60-296)107 (54-196)360 (68-607)< 0.001
Total bilirubin (NV ≤ 1 mg/dL)1.7 (0.6-4.5)2.7 (0.6-5.3)1.2 (0.7-2.5)0.05
ALP (NV ≤ 350 U/L)296 (204-469)283 (199-462)301 (242-494)0.328
ALP/AST ratio0.7 (0.3-2.2)0.4 (0.2-1.6)2.3 (0.7-3.5)0.002
Albumin (NV: 30-50 g/dL)42 (38-44)42 (37-44)42 (40-46)0.082
INR (NV: 0.9-1.2)1.2 (1.1-1.5)1.2 (1.1-1.6)1.1 (1.0-1.2)< 0.05
Platelet (109/L)252 (180-350)234 (167-314)319 (251-393)< 0.05
Autoimmune profile
ANA (≥ 1:20): n (%)55 (73)38 (70)17 (81)0.777
SMA (≥ 1:20): n (%)53 (71)38 (70)15 (71)1
Anti-LKM-1 (≥ 1:10): n (%)12 (16)12 (22)0 (0)< 0.05
Anti-LC1: n (%)9 (12)9 (17)0 (0)< 0.05
ANCA: n (%)37 (49)22 (41)15 (71)< 0.05
IgG g/dL (NV: 0.5-1.8 g/dL)2.0 (1.4-3.2)2.3 (1.4-3.3)1.7 (1.5-2.2)0.325
IgG > ULN: n (%)51 (68)37 (69)14 (67)1
Histology, n (%)
Interface hepatitis51 (68)42 (78)12 (57)0.09
Fibrosis61 (81)42 (78)19 (90)0.324
Cirrhosis17 (23)15 (28)2 (10)0.127
Features of biliopathy162 (83)37 (68)17 (81)0.764
Table 2 Response to medical treatment and outcome of 75 patients with autoimmune liver diseases
Variables
All patients, n = 75
AIH, n = 54
ASC, n = 21
P value
Treatment, n
Prednisone alone26 (35%)19 (35%)7 (33%)1
Prednisone + Azathioprine30 (40%)28 (52%)2 (10%)< 0.001
Prednisone + MMF5 (7%)3 (5%)2 (10%)0.615
Prednisone + Vancomycin4 (5%)1 (2%)3 (14%)0.064
Prednisone + Azathioprine + Vancomycin 2 (3%)0 (0%)2 (10%)0.075
Prednisone + MMF + Vancomycin6 (8%)1 (2%)5 (23%)0.005
Prednisone + Tacrolimus1 (1%)1 (2%)0NA
Cyclosporine1 (1%)1 (2%)0NA
Response to treatment
Normal AST (NV ≤ 45 U/L): n69 (92%)52 (96%)17 (81%)0.048
Time to normalize AST (yr)0.1 (0.1-0.5)0.2 (0.1-0.6)0.1 (0.1-0.2)0.19
GGT (< 50 UI/L), n68 (91%)51 (94%)17 (81%)0.811
Time to normalize GGT (yr)0.3 (0.2-0.6)0.3 (0.2-0.5)0.3 (0.2-1.1)0.062
Immunological remission1: n25 (33%)22 (40%)3 (14%)0.032
Time to immunological remission3.1 (2.2-4.2)3.8 (2.9-4.3)3.4 (3.2-3.7)0.86
Relapse of AILD during treatment, n
At least one relapse36 (48%)22 (41%)14 (67%)< 0.070
1 relapse alone26 (35%)17 (31%)9 (43%)0.421
≥ 2 relapses10 (13%)5 (9%)5 (24%)0.131
Outcome at last follow up
Median follow up, yr (range)4.4 (0.6-13.8)4.1 (1.2-11.7)4.5 (0.6-13.8)0.079
Alive75 (100%)54 (100%)21 (100%)NA
OFF-IS therapy6 (8%)5 (9%)1 (5%)0.666
Medical treatment68 (91%)49 (91%)19 (90%)1
Liver transplant1 (1%)0 (0%)1 (5%)0.28
Table 3 Demographic, biochemical and histological features of 12 patients with autoimmune liver disease treated with oral vancomycin
Patients/ diagnosis
Gender
Age at diagnosis (yr)
Type of presentation
IBD
Splenomegaly1
IgG > ULN
Auto-antibodies
Histology
Medications
Interface hepatitis
Fibrosis
Cirrhosis
Biliopathy3
AIHF4.2AsymptomaticIC NotYesSMA 1:40; p-ANCANoYesNoYesPred/MMF/UDCA/Mesa
AIHF10.9AsymptomaticUCNotYesANA 1:320; SMA 1:160; p-ANCA positive YesYesNoYesPred/UDCA/Mesa
ASCF16InsidiousNoneYesNotANA 1:160; p-ANCA positiveYesYesYesNoPred/MMF/UDCA
ASCM4.3AsymptomaticCDNotYesANA 1:160; SMA 1:160; p-ANCA +++YesYesNoYesPred/UDCA/Mesa
ASCF8.6InsidiousUCNot NotSMA 1:40; p-ANCA positiveNoNoNoYesPred/AZA/UDCA/Mesa
ASC F12.1InsidiousUC2NotNotSMA 1:40; p-ANCA positiveNoNoNoYesPred/AZA/UDCA
ASC M14.1InsidiousNoneNotNotSMA 1:40; p-ANCA positiveYesYesNoYesPred/AZA/UDCA
ASC M14.3AcuteUCNotYesANA 1:320; SMA 1:320YesNoNoYesPred/UDCA/Mesa
ASCF13.8AsymptomaticUCYesYesANA 1:640; p-ANCA positiveNoYesYesYesPred/MMF/UDCA/Mesa
ASCF5.1AcuteICNotNotANA 1:160; p-ANCA positiveNoYesNoYesPred/MMF/UDCA/Mesa
ASCF13.1AcuteCDYes YesANA 1:80; p-ANCA positiveNoYesNoYesPred/MMF/UDCA/Mesa
ASCF3.6AsymptomaticUCYesNotANA 1:160; SMA 1:80; p-ANCA positiveYesYesNoYesPred/MMF/UDCA/Mesa
Table 4 Biochemical response to oral vancomycin and Sclerosing Cholangitis Outcomes in Pediatrics index score of 12 patients with autoimmune liver disease treated with oral vancomycin
Patients/diagnosis
Age at OVT (yr)
AST (NV ≤ 45 U/L)
ALT (NV ≤ 45 U/L)
GGT (NV ≤ 50 U/L)
Response to OVT 1SCOPE index score2
Time on OVT (mo)
OVT side-effect
Overall FU3 (mo)
Before OVT
After OVT
TTN
Result
Before OVT
After OVT
TTN
Result
Before OVT
After OVT
TTN
Result
Before OVT
After OVT
AIH5.4212394 moNV147176 moNV73226 moNVComplete3 low risk0 low risk99None113
AIH11.8251312 moNV359399 moNV26348 moNVComplete3 low risk0 low risk72None73
ASC16.898473 mo< 1.5 NV140704< 1.5 NV39834< 1.5 NVPartial8 high risk8 high risk16None26
ASC4.886287 dNV1563814 dNV844414 dNVComplete4 medium risk1 low risk37None39
ASC13.160141 moNV365383 moNV68274 moNVComplete 5 medium risk2 low risk31None84
ASC15.2714014 moNV1405614 mo< 1.5 NV5216412 mo-None6 high risk6 high risk6None68
ASC17.4113651 mo< 1.5 NV2051411 mo-492261 mo-None6 high risk4 medium risk3None52
ASC15407306 moNV856356 moNV61281 moNVComplete5 medium risk2 low risk40None49
ASC17.3102372 moNV111362 moNV135825 mo< 1.5 NVPartial6 high risk4 medium risk18None61
ASC12.576312 moNV124407 moNV86; TX426 moNVComplete5 medium risk4 medium risk47None135
ASC13.9123155--165154--1651800--None8 high risk8 high risk6None86; TX
ASC13.2141135--156180--71 mo (range 26-165)136--None7 high risk7 high risk4None165
Response to OVT10/12 (83%)9/12 (75%)8/12 (67%)Median: 34 (range 1-99)71 (range 26-165)