Clinical characteristics and corticosteroid therapy in patients with autoimmune-hepatitis-induced liver failure

Table 1 Clinical features of patients at the time of diagnosis

Case	Age/sex (yr)	TBil (mg/dL)	PTA	ALT (IU/L)	ALP (IU/L)	Creatinine (mg/dL)	MELD	Liver cirrhosis	HE	SIRS	Liver failure type
1	36/F	13.9	28%	300	185	0.77	22	Yes	No	No	ACLF
2	50/F	10.1	27%	326	107	0.63	29	Yes	No	No	ACLF
3	66/F	21.3	12%	452	275	0.77	30	Yes	No	Yes	ACLF
4	48/F	26.6	38%	374	158	1.37	27	Yes	No	No	ACLF
5	44/F	21.7	32%	280	134	0.83	32	No	Yes	No	Subacute
6	42/M	32.8	39%	791	144	1.32	31	No	Yes	Yes	Subacute
7	33/F	29.0	30%	139	148	0.93	24	No	No	No	Subacute
8	19/F	15.6	33%	234	439	2.87	36	Yes	No	Yes	ACLF
9	67/M	25.5	19%	145	54	0.87	29	Yes	Yes	No	ACLF
10	58/F	27.9	36%	274	212	1.23	30	No	Yes	No	Subacute
11	59/F	24.0	20%	138	150	1.50	41	Yes	No	No	ACLF
12	75/F	19.6	30%	725	173	0.86	23	Yes	No	No	ACLF
13	56/F	29.8	31%	268	420	0.80	31	No	Yes	No	Subacute
14	41/F	32.5	5%	99	81	1.87	44	Yes	Yes	No	ACLF
15	72/M	27.4	19%	400	195	1.54	38	No	Yes	No	Acute
16	45/F	11.1	39%	512	162	0.58	22	Yes	No	No	ACLF
17	45/F	19.0	38%	557	290	0.87	22	No	No	No	Subacute
18	39/F	23.0	26%	147	35	0.75	21	Yes	Yes	No	ACLF
19	72/F	23.3	23%	337	123	1.41	36	Yes	Yes	No	ACLF
20	52/F	17.1	38%	197	168	0.76	19	No	No	No	Subacute
21	65/F	17.7	35%	134	32	0.66	19	No	No	Yes	Subacute
22	40/F	27.1	31%	76	92	1.67	31	Yes	No	No	ACLF

HE: Hepatic encephalopathy; SIRS: Systemic inflammatory response syndrome; TBil: Total bilirubin; PTA: Prothrombin activity; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase; MELD: Model for end-stage liver disease; ACLF: Acute on chronic liver failure.

Table 2 Immunoserological features and outcomes of enrolled patients

Case	ANA (fold)	ASMA (fold)	LKM-1 (fold)	AMA	IgG (mg/dL)	AIH score	Simplified AIH score	Corticosteroid therapy	Outcome	Weeks from onset to death
1	> 1000	-	-	-	2807	17	6	Yes	Survival	/
2	640	-	-	-	5140	16	6	No	Death	11
3	> 1000	-	UD	-	36%	15	6	No	Death	10
4	> 1000	-	UD	-	1140	13	4	No	Death	11
5	640	-	-	-	20%	13	4	No	Death	3
6	640	80	320	-	1300	11	6	No	Death	6
7	640	-	-	-	2118	15	6	Yes	Survival	/
8	> 1000	160	-	-	54%	15	6	No	Death	5
9	> 1000	-	-	-	30%	12	6	No	Death	5
10	> 1000	1000	UD	-	2026	14	6	No	Death	8
11	> 1000	-	-	-	2358	14	6	No	Death	3
12	> 1000	-	UD	-	1983	14	6	Yes	Death	22
13	> 1000	-	-	-	3014	15	6	No	Death	2
14	> 1000	320	-	-	2167	14	6	No	Death	3
15	> 1000	-	UD	-	UD	12	4	No	Death	2
16	> 1000	160	UD	-	2823	15	4	Yes	Death	19
17	> 1000	-	-	-	23%	13	5	No	Death	6
18	> 1000	-	-	-	3553	18	6	Yes	Survival	/
19	> 1000	1000	-	-	3474	19	8	Yes	Death	11
20	640	-	160	-	1456	14	6	Yes	Survival	/
21	640	-	-	-	1768	14	6	No	Death	12
22	1000	-	-	-	2776	15	6	No	Death	8

Cases 3, 5, 8, 9 and 17 presented percentage of γ-globulins. UD: Undetermined. ANA: Anti-nuclear antibody; ASMA: Anti-smooth muscle antibody; LKM: Liver kidney microsomal antibody; AMA: Antimitochondrial antibody; AIH: Autoimmune hepatitis.

Table 3 Possible factors associated with survivors and non-survivors

Variables	Survivor (n = 4)	Non-survivor (n = 18)	P value
Age (yr)	40.00 ± 8.37	53.56 ± 14.58	0.0411
SIRS (yes), n (%)	0 (0)	4 (22.2)	-
Cirrhosis (yes), n (%)	2 (50)	11 (61)	0.6852
Acute/subacute/ACLF	0/2/2	1/6/11	-
TBil (mg/dL)	20.75 ± 6.67	22.94 ± 6.58	0.4961
ALT (IU/L)	195.75 ± 74.09	340.11 ± 205.69	0.2681
PTA (%)	30 .50 ± 5.26	28.17 ± 9.87	0.9961
MELD score	21.50 ± 2.08	30.61 ± 6.70	0.0151
Hepatic encephalopathy (yes), n (%)	1 (25)	8 (44)	0.4632
Ascites (yes), n (%)	2 (50)	14 (78)	0.2802
ANA (> 1:100)	4 (100)	18 (100)	-
IgG (mg/dL)	2483.50 ± 901.45	2344.88 ± 1119.46	0.5911
Corticosteroid treatment	4 (100)	3 (16.7)	0.0012
Plasma exchange, n (%)	1 (25)	3 (16.7)	1.0002

¹Mann-Whitney test;

²χ² test. ANA: Anti-nuclear antibody.

Table 4 Clinical characteristics of patients receiving corticosteroid treatment

Case	Treatment	Loading dose of corticosteroid (mg/d)	Duration (d)1	MELD2	Hepatic encephalopathy2	Creatinine (mg/dL)2	Adverse events	Outcome
1	mPSL	30	16	23	None	0.77	None	Survival
7	PSL	50	26	24	None	0.93	Oral fungal infection	Survival
18	mPSL	32	35	19	None	0.75	Oral fungal infection	Survival
20	mPSL	32	21	20	None	0.76	Oral fungal infection, pulmonary fungal infection	Survival
12	mPSL	24	105	25	None	0.86	Oral fungal infection, pulmonary fungal infection	Death
16	mPSL	24	101	26	None	0.58	Oral fungal infection	Death
19	PSL	30	67	36	Yes	1.41	Oral fungal infection	Death

¹Days from diagnosis to corticosteroid therapy;

²At corticosteroid application. PSL: Prednisolone; mPSL: Methylprednisolone.