Optimizing management in autoimmune hepatitis with liver failure at initial presentation

Table 1 Clinical characteristics of patients with autoimmune hepatitis with liver failure at initial presentation

	Villamil et al[22]1(n = 28)	Kessler et al[23](n = 10)	Miyake et al[24](n = 11)	Ichai et al[25](n = 16)	Verma et al[7](n = 20)
Study design	Retrospective	Retrospective	Retrospective	Retrospective	Retrospective
Age (yr)2	41	40 ± 15.9	53 (16-75)	36 ± 13.1	41.3 ± 14.2
Definition of LF	NA	NA	PT < 40% and HE ≥ grade 2	HE within 12 wk of jaundice	Any grade HE and/or INR > 2
Symptoms duration2	NA	3.2 wk	24 (16-52) d	NA	2.1 ± 2.5 mo3
Female	NA	8 (80%)	11 (100%)	14/16 (87.5%)	15 (75%)
Ethnicity or country of origin	South American	80% White	Japanese	French	70% black
Definite/probable AIH (IAIHG4 criteria)	NA	NA5	3(36%)/8 (64%)	NA	9(45%)/11(55%)
LC/LKM6 positive ANA/SMA7 positive	6 (21.4)	1 (10%)		3 (18.7%)	NA
	22 (78.5%)	7 (70%)	NA	11 (68.7%)	20 (100%)
Bilirubin2 (mg/dL)	3988	16.97 ± 9.83	20.6 (5.9-31)	425 (278-850)8	19.3 ± 10.3
AST or ALT2	NA	1179 ± 1127.17	220 (59-1094)	678 (60-2867)	1147.1 ± 711.4
INR2 or PT	30%	49.3 ± 66.9	29% (6%-38%)	5.36 (1.7-12.2)	2.7 ± 1.4
HE9 at onset	28 (100%)	8 (80%)	11 (100%)	10 (62.5%)	19 (95%)
Cirrhosis	None	2/10 (20%)	NA	None	8/20 (40%)
MELD2	NA	NA	NA	37 (24-47)	28 ± 7.41
Sub-massive or massive necrosis (SMN, MN)	19/23 (82.6%)	5/10 (50%)	NA	16/16 (100%) 15 needed LT and/or died	12/19 (63.1%), 10 needed LT and or died
	17 needed LT and/or died
Immunosuppressant regimen used	Prednisone 60 mg/d	Corticosteroids (Dose NA) and other10	Prednisolone	Prednisone 1 mg/kg per day and other10	Corticosteroids11
			40-60 mg/d and steroid pulse		20-1250 mg/d
Poor prognostic criteria	1: PT < 20%; 2: Grade 4 HE; 3: SMN at diagnosis; 4: 20% increase in PT at day 3 of steroids	NA	1: High bilirubin at onset; 2: Worsening bilirubin during days 8-15 of steroid therapy	NA	1: Absence of cirrhosis; 2: MELD > 28; 3: Worsening trend in bilirubin and INR after 3.7 ± 0.6 d of steroid therapy
Septic events	NA	NA	NA	7 (43.7%), of whom 6 had received steroids	2 (10%), of whom 1 received steroids

¹Published only in abstract form;

²Data presented as mean ± SD or median (range);

³Duration from first symptom (and not necessarily jaundice/hepatic encephalopathy) to hospitalization;

⁴IAIHG: International Autoimmune Hepatitis Group;

⁵Met IAIHG criteria, data on probable or definite disease unavailable;

⁶LKM/LC: Liver kidney microsomal antibody/liver cytosol antibody;

⁷ANA/SMA: antinuclear antibody/anti-smooth muscle antibody;

⁸Values in µmol/L;

⁹HE: Hepatic encephalopathy;

¹⁰Additional immunosuppression was used in nine patients in the study of Kessler et al (azathioprine, tacrolimus, mycophenolate mofetil, 6-mercaptopurine, cyclosporine) and in one patient in the study of Ichai et al (azathioprine and cyclosporine);

¹¹Included prednisone, hydrocortisone and methylprednisone, (converted to equivalent doses of prednisone); LT:Liver transplantation; PT: Prothrombin time; AIH: Autoimmune hepatitis.

Table 2 Outcomes of patients with autoimmune hepatitis and initial presentation with liver failure

Study	Villamil et al[22](n = 28)	Kessler et al[23](n = 10)	Miyake et al[24](n = 11)	Ichai et al[25](n = 16)	Verma et al[7](n = 20)
Treated with IS1	25	10	8	12	14
Responders to steroids	9 (36%) (alive)	4 (40%) (alive)	2 (25%) (alive)	1 (8.3%) (alive)	7 (50%) (alive)
Non responders	16	6	6	11	7
LT	11 (2 Died)	3	1	10 (1 Died)	1 (Died)
Listed for LT	-	1	-	-	1 (Died)
Died without LT	5	2	5	14	52
Not treated with IS1	3	-	33	4	6
Spontaneous survival	-	-	3	-	-
LT	1	-	-	3	5 (1 Died)
Listed for LT	-	-	-	-	-
Died	2	-	-	1	1
Overall underwent LT or listed for LT	12/28 (42.8%)	4/10 (40%)	1/11 (9%)	13/16 (81.2%)	7/20 (35%)
Overall mortality	9/28 (32.1%)	2/10 (20%)	5/11 (45.4%)	3/16 (18.7%)	9/20 (45%)

¹IS: Immunosuppression;

²Four died while being evaluated for liver transplantation, in 1 sepsis precluded liver transplantation evaluation;

³Treated with plasmapheresis and or stronger neo-minophagen;

⁴Not evaluated for LT due to sepsis; LT: Liver transplantation. Additional outcome data obtained by personal communication with authors.