Clinical Characteristics and Predictors of Cirrhosis in Autoimmune Hepatitis: Insights from an Austrian Cohort

Introduction Autoimmune hepatitis (AIH) is a rare aetiology of chronic liver disease, with a variable clinical presentation and course. Systematic clinical data of Austrian patients with AIH are lacking.

Material and Methods This retrospective analysis aims to assess clinical characteristics, treatment, and predictors of cirrhosis in patients with AIH from a tertiary centre in Austria. Multivariable logistic regression was used to identify risk factors ([Table 1]).

Table 1 shows the epidemiological characteristics of the study population, including diagnostic methods, AIH-associated diseases, comorbidities, and overlap syndromes. P-values indicate differences between the group with cirrhosis and the group without cirrhosis.

Table 1.:	All patients (N=99)	Group with cirrhosis (N=31)	Group without cirrhosis (N=67)	p-value
Age at Diagnosis [years (SD)]	53.5 (15.7)	61.5 (12.3)	50.7 (15.9)	p=<0.01
Sex (female) [n (%)]	77 (77.8)	24 (77.4)	52 (77.6)	n.s
Years of follow up [years (IQR 25;75)]	4.7 (5.0)	5.0 (5.5)	4,6 (5.0)	n.s
BMI [kg/m² (SD)]	28.7 (10.9)	28.1 (4.4)	29,0 (12.6)	n.s
AIH-Associated Diseases [n (%)]	31 (31)	11 (35)	20 (30)	n.s
Asthma [n (%)]	2 (2)	0	2 (3)	n.s
Hashimoto thyroiditis [n (%)]	17 (17)	8 (26)	9 (13)	n.s
Diabetes mellitus type 1 [n (%)]	1 (1)	1 (3)	0	n.s
Ulerative colitis [n (%)]	1 (1)	0	1 (1)	n.s
Celiac disease [n (%)]	2 (2)	1 (3)	1 (1)	n.s
Rheumatoid arthritis [n (%)]	2 (2)	1 (3)	1 (1)	n.s
Sjögren syndrome [n (%)]	3 (3)	0	3 (4)	n.s
MTCD [n (%)]	0 (0)	0	0	-
SLE [n (%)]	2 (2)	0	2 (3)	n.s
Psoriasis [n (%)]	1 (1)	0	1 (1)	n.s
Comorbidities [n (%)]	44 (44)	13 (42)	25 (37)	n.s
Diabetes mellitus type 2-3 [n (%)]	14 (14)	6 (19)	8 (12)	n.s
Coronary heart disease [n (%)]	8 (8)	4 (13)	4 (6)	n.s
Stroke [n (%)]	1 (1)	1 (3)	0	-
Arterial hypertension [n (%)]	28 (28)	11 (35)	17 (25)	n.s
Overlap [n (%)]	21 (21)%	6 (19)	15 (22)	n.s
PBC [n (%)]	13 (13)	3 (10)	10 (15)	n.s
PSC [n (%)]	7 (7)	3 (10)	4 (6)	n.s
PBC/PSC [n (%)]	1 (1)	0	1 (1)	n.s
Treatment
AZA+Corticosteroids [n (%)]	60 (60.6)	15 (48.4)	45 (67.2)	n.s.
Corticosteroids monotherapy [n (%)]	12 (12.1)	4 (12.9)	8 (11.9)	n.s
AZA monotherapy [n (%)]	8 (8.1)	3 (9.7)	5 (7.5)	n.s
No medication/documentation [n (%)]	19 (19.2)	9 (29.0)	9 (13.4)	p=0.04
Medication changes [n (%)]	19 (19.2)	4 (13.0)	15 (22.4)	p=0.03
Outcome
Death [n (%)]	9 (9.0)	6 (19.4)	2 (3.0)	p<0.01
Carcinoma [n (%)]	1 (1.0)	1 (3.2)	0	-
Varices [n (%)]	22 (22.2)	20 (64.5)	2 (3.0)	p<0.01
Variceal Bleeding [n (%)]	2 (2.02)	2 (6.5)	0	-
LTx [n (%)]	4 (4.0)	3 (16.1)	1 (1.5)	n.s.

Abbreviations: AIH=Autoimmune Hepatitis, PBC=Primary Biliary Cholangitis, PSC=Primary Sclerosing Cholangitis, MTCD=Mixed Connective Tissue Disease, SLE=Systemic Lupus Erythematosus, DM=Diabetes Mellitus, KHK=Coronary Heart Disease, HTN=Hypertension, AZA=Azathioprine, LTx=Liver Transplantation, BMI=Body Mass Index, Std=Standard Deviation,IQR=Interquartile Range, n.s.=not significant

Results A total of 99 patients were included (mean age of 53.5 years [SD: 15.7], 78% female). Median time after AIH diagnosis at baseline was 5.7 (IQR: 2.5;7.2) years. AIH-associated comorbidities were diagnosed in 32.3% of patients (most commonly Hashimoto thyroiditis: 17.2%). Additionally, 44.4% of patients with AIH had cardiometabolic diseases. Regarding outcomes, cirrhosis was diagnosed in 31 (31%) patients. In both groups (cirrhosis vs. non-cirrhosis), the initial treatment mainly consisted of corticosteroids with azathioprine (AZA) (total: 60%, cirrhosis: 48%, non-cirrhosis: 67%, p>0.05). Used treatment regimen was undocumented in 19% of cases. A switch to alternative immunosuppressants or a change to corticosteroid monotherapy was observed in 19 (19,2%) patients (Table 2). Higher age at the time of diagnosis was associated with an increased risk for cirrhosis (OR=1.063, 95% CI: [1.02, 1.10], p=<0.01), while follow-up duration, sex, BMI, and AIH-associated diseases were not associated with an increased risk for developing cirrhosis. Furthermore, treatment changes (12.9% vs 22.4%, p=0.03) as well as continuous treatment documentation (13.4% vs. 29.0%, p=0.04) were associated with better outcomes. Mortality was significantly higher in the group with cirrhosis (19.3% vs 2.9%, p<0.01).

Conclusion Age at diagnosis is significantly associated with cirrhosis risk, highlighting the importance of a timely AIH diagnosis and treatment for patient prognosis. Changes of treatment regimens were associated with a decreased risk for cirrhosis. Future research on documentation quality and comparison to larger cohorts is warranted.

Publication History

Article published online:
13 May 2025