Dataset Information

Role of age in presentation, response to therapy and outcome of autoimmune hepatitis.

ABSTRACT:

Background

Few studies with diverging results and a small sample size have compared autoimmune hepatitis (AIH) in the elderly to younger patients.

Aim

To unbiasedly investigate the role of age in behaviour and treatment outcome of AIH.

Methods

All patients with probable or definite AIH type 1 in four tertiary academic centres were included in this retrospective-and since 2006 prospective-cohort study. Influence of age on presentation, remission and outcome of AIH were investigated.

Results

359 patients were included. Presence of cirrhosis at AIH diagnosis around 30% was independent of age. ALAT was higher at age 30-60 years on AIH diagnosis, and above age 60 there were less acute onset, less jaundice and more concurrent autoimmune disease. Remission was reached in 80.2%, incomplete remission in 18.7%, only 1.1% (all aged 50-65) was treatment-refractory. Age was not an independent predictor of remission, while cirrhosis was. Above age 45 there was more diabetes, above age 60 more loss of remission. Rate of progression to cirrhosis was 10% in the 10 years after diagnosis and unrelated to age at AIH diagnosis. With onset below age 30, there was more development of decompensated cirrhosis over time. With higher age at AIH diagnosis there was a lower survival free of liver-related death or liver transplantation.

Conclusions

AIH presents at all ages. Age influences features at diagnosis, but not response to treatment, while survival without liver-related death or liver transplantation decreases with higher age at diagnosis.

SUBMITTER: Baven-Pronk MAMC

PROVIDER: S-EPMC6026593 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Publications

Role of age in presentation, response to therapy and outcome of autoimmune hepatitis.

Baven-Pronk Martine A M C MAMC Biewenga Maaike M van Silfhout Joanne J JJ van den Berg Aad P AP van Buuren Henk R HR Verwer Bart J BJ van Nieuwkerk Carin M J CMJ Bouma Gerd G van Hoek Bart B

Clinical and translational gastroenterology 20180702 6

<h4>Background</h4>Few studies with diverging results and a small sample size have compared autoimmune hepatitis (AIH) in the elderly to younger patients.<h4>Aim</h4>To unbiasedly investigate the role of age in behaviour and treatment outcome of AIH.<h4>Methods</h4>All patients with probable or definite AIH type 1 in four tertiary academic centres were included in this retrospective-and since 2006 prospective-cohort study. Influence of age on presentation, remission and outcome of AIH were inves ...[more]

PMID: 29961755

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Project description:Autoimmune hepatitis (AIH) is a chronic hepatitis with an increasing incidence. The majority of patients require life-long immunosuppression and incomplete treatment response is associated with a disease progression. An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. We assessed the capacity of baseline parameters including the iron status to predict the treatment response upon standard therapy in 109 patients with untreated AIH type 1 (AIH-1) in a retrospective single center study. Thereby, a hyperferritinemia (> 2.09 times upper limit of normal; Odds ratio (OR) = 8.82; 95% confidence interval (CI): 2.25-34.52) and lower immunoglobulins (<1.89 times upper limit of normal; OR = 6.78; CI: 1.87-24.59) at baseline were independently associated with the achievement of complete biochemical remission upon standard therapy. The predictive value increased when both variables were combined to a single treatment response score, when the cohort was randomly split into a training (area under the curve (AUC) = 0.749; CI 0.635-0.863) and internal validation cohort (AUC = 0.741; CI 0.558-0.924). Patients with a low treatment response score (<1) had significantly higher cumulative remission rates in the training (p<0.001) and the validation cohort (p = 0.024). The baseline hyperferritinemia was accompanied by a high serum iron, elevated transferrin saturations and mild hepatic iron depositions in the majority of patients. However, the abnormal iron status was quickly reversible under therapy. Mechanistically, the iron parameters were not stringently related to a hepatocellular damage. Ferritin rather seems deregulated from the master regulator hepcidin, which was down regulated, potentially mediated by the elevated hepatocyte growth factor. In conclusion, baseline levels of serum ferritin and immunoglobulins, which are part of the diagnostic work-up of AIH, can be used to predict the treatment response upon standard therapy in AIH-1, although confirmation from larger multicenter studies is pending.

Dataset Information

Role of age in presentation, response to therapy and outcome of autoimmune hepatitis.

Background

Aim

Methods

Results

Conclusions

Publications

Role of age in presentation, response to therapy and outcome of autoimmune hepatitis.

Similar Datasets

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