Table 1.

A summary of corticosteroid responses from previous studies on acute severe presentations of AIH – the ‘sicker’ cohorts had poorer response to corticosteroids.

Study	No. of patients	Definition of AS-AIH or AIH-ALF	Bilirubin of whole cohort	INR/PT of whole cohort	MELD of whole cohort	Corticosteroid therapy n (%)	Corticosteroid dose	HE prior to corticosteroid therapy n (%)	Outcome in corticosteroid cohort n (%)
Zachou et al. 2019	34	•Acute hepatitis (<24 weeks) •No HE •Transaminases >10 × ULN •Bilirubin >4 mg/dl •INR ≥1.5 •No histological lesions of chronic disease	Median 173 μmol/L (54–619)	Median INR 1.52 (1.5-2.27)	Median 18 (12–24)	34/34 (100%)	Induction regimens: Prednisolone 1.5 mg/kg/day (intravenous) or 3 days of Methylprednisolone 1 g/day with subsequent Prednisolone dose of 63.2 ±13.7 mg/day (intravenous)	0	•33 (97%) responded to corticosteroids •1 died on waiting list for LT
Anastasiou et al. 2018	32	•Acute presentation (<4 weeks from symptom onset) of HE (any degree) •INR ≥1.5 •No apparent pre-existing liver disease at admission •Advanced fibrosis/cirrhosis not excluded	Median 275 μmol/L (188)	Median INR 1.7 (0.6)	Median 22 (5)	32/32 (100%)	Average Prednisolone dose 153.9 mg/day (60–500 mg) (oral or intravenous)	?All (100%)	•?29 (91%) responders to corticosteroids •1 LT •2 died
de Martin et al. 2017	121	•Acute presentation •Histological features of AIH •INR ≥1.5 ± total bilirubin >200 μmol/L •Advanced fibrosis/ cirrhosis not excluded (52% with F0-F1)	Median 252 μmol/L (188-376)	Median INR 1.9 (1.5-2.8)	Median 25 (21-28)	110/121 (91%)	Corticosteroid dose of 1 mg/kg/day (unspecified route)	8/110 (7%)	•77 (70%) responded to corticosteroids •32 LT •1 died
Moenne-Loccoz et al. 2016	17	•Acute presentation (<26 weeks) •INR ≥1.5 •No histological lesions of chronic disease	Median 429 μmol/L (106–797)	Median INR 2.3 (1.5-5.5)	Median 26 (20–51)	15/17 (88%)	Majority Prednisolone, 3 treated with Methylprednisolone Initial median dose of 60 mg/day (40-100 mg) (unspecified route)	?1 or 2/15 (7 or 13%)	•9 (60%) responded to corticosteroids •6 LT
Fujiwara et al. 2016	20	•Acute presentation: Severe hepatitis (no HE); Fulminant hepatitis (>grade 2 HE within 8 weeks of symptoms onset); Late-onset hepatic failure (HE development between 8 and 24 weeks after symptom onset) •PT <40%	Mean 304 μmol/L (SD ± 160)	Mean PT 29% (SD ± 13)	Mean 27 ± 7	19/20 (25%)	Prednisolone 40-60 mg/day (unspecified route) or Methylprednisolone 500-1000 mg/day (unspecified route)	14/19 (74%)	Severe hepatitis: •5/5 (100%) responded to corticosteroids Fulminant hepatitis: •2/13 (15%) responded to corticosteroids •3 LT •8 died without LT Late-onset hepatic failure: •1/2 (50%) responded to corticosteroids •1 LT
Yeoman et al. 2014	32	•Acute presentation (<26 weeks) •INR ≥1.5 •No histological lesions of chronic disease	Median 463 μmol/L (55-1,208)	Median INR 2.2 (1.5-3.5)	Median 29 (22-40)	23/32 (72%)	Prednisolone 20-40 mg/day (oral) or Hydrocortisone 100 mg TDS (intravenous)	5/23 (21%) grade 1-2	•10 (43%) responded to corticosteroids •2 responded to additional immunosuppression •10 LT
Mendizibal et al. 2015	40	•Acute presentation (<26 weeks) of HE (any degree) since onset of first symptoms •INR ≥1.5 or PT <50% •No known underlying liver disease, but only 11 patients with no fibrosis on biopsy	Median 361 μmol/L ±133	Median INR 2.92 ± 1.45	Median 29 ± 5	17/40 (43%)	Meprednisone 40-60 mg/day (unspecified route)	4 /17 (24%) ≥ grade 3	•7 (41%) responded to corticosteroids •8 LT •2 died from pneumonia
Karkhanis et al. 2014	66	•Acute presentation •HE (any degree) •INR ≥1.5 •Each centre determined diagnosis of AIH, some reclassified after histology review •Fibrosis/cirrhosis stage not discussed	Mean 397 μmol/L (SD ± 156)	Mean INR 3.33 (SD 3.17)	Mean 30.9 (SD 9.5)	25/66 (38%)	Mean prednisone dose 42.5 mg/day (unspecified route)	25/25 (100%)	•8 (32%) spontaneous survival •?7 (28%) LT •?10 (40%) died
Ichai et al. 2007	16	•Acute hepatitis with ALF/HE <2 weeks (fulminant) or 2-12 weeks (sub-fulminant) after onset of jaundice •No histological lesions of chronic disease	Median 425 μmol/L (278-850)	Median INR 5.36 (1.7-12.2)	Median 37 (24-47)	12/16 (75%)	Mean prednisone dose 1.3 mg/kg/day (intravenous)	8/12 (67%) grade 1: 1 grade 2: 2 grade 3: 3 grade 4: 2	•1 (8%) responded to corticosteroids •10 LT •1 died of sepsis

? not clear in study report.

AIH, autoimmune hepatitis; ALF, acute liver failure; ALI, acute liver injury; AS-AIH, acute severe autoimmune hepatitis; HE, hepatic encephalopathy; IAIHG, International Autoimmune Hepatitis Group; INR, international normalised ratio; LT, liver transplantation; MELD, model for end-stage liver disease; PT, prothrombin time; ULN, upper limit of normal.