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

Baven-Pronk, Martine; Biewenga, Maaike; Silfhout, Joanne J. van; Berg, Aad P. van den; Buuren, Henk R. van; Verwer, Bart J.; Nieuwkerk, C.M.J. van; Bouma, Gerd; Hoek, Bart van

doi:10.1038/s41424-018-0028-1

Cited by 27 publications

(29 citation statements)

References 29 publications

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“…Knowledge about clinical characteristics that have a significant impact on treatment outcome provides useful information to the concerned physician in the tailoring of management strategies of patients with AIH. [7,8] Poor response to treatment in patients with AIH may lead to cirrhosis and its life threatening complications. It is, therefore, essential to study those patients and the disease related characteristics that adversely affect the treatment outcome so that careful monitoring of such patients can be performed and alternative treatment strategies be anticipated.…”

Section: Introductionmentioning

confidence: 99%

Autoimmune hepatitis: Clinical characteristics and predictors of biochemical response to treatment

Tasneem

Luck

2020

Journal of Translational Internal Medicine

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Background and ObjectivesAutoimmune hepatitis (AIH) is an important cause of chronic liver disease. Aim of this study was to evaluate the clinical characteristics and factors predicting response to treatment in patients with AIH.MethodsIn this prospective observational study, all patients diagnosed with AIH from 2017 to 2019 were included. Biochemical response to the treatment was checked three months after the start of the treatment. Response was considered good if transaminases normalized, or poor if either remained persistently elevated or improved partially.ResultsOf the total 56 patients, 41 (73.2%) were females. Mean age was 29.5 (±16.9) years. About half (53.6%; n = 30) the patients were aged < 25 years and majority [47 (83.9%)] were cirrhotic. Autoimmune serology was negative in 20 (35.7%). Seronegativity was associated with severe necroinflammation (P = 0.015) and esophageal varices (P = 0.021). Response to treatment was good in 34 (60.7%). Bivariate analysis showed that good response to treatment was associated with pre-treatment serum IgG level > 20 g/L (P = 0.024), presence of pseudorosettes on histopathology (P = 0.029) and three months post-immunosuppression serum total bilirubin < 2mg/dL (P < 0.001). Multivariate logistic regression analysis showed that only pre-treatment serum IgG >20 g/L (P = 0.038) and post-treatment serum total bilirubin <2 mg/dL (P = 0.004) were independent predictors of good response to treatment.ConclusionMajority of AIH patients in our study were young and cirrhotic. A negative autoimmune serology does not rule out AIH and liver biopsy may be required to confirm the diagnosis. Seronegative AIH rapidly progresses to advanced liver disease. Response to treatment is good with pre-treatment IgG > 20g/L and post-treatment total bilirubin < 2 mg/dL.

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Section: Introductionmentioning

confidence: 99%

Autoimmune hepatitis: Clinical characteristics and predictors of biochemical response to treatment

Tasneem

Luck

2020

Journal of Translational Internal Medicine

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“…In approximately 30% of the patients, cirrhosis was present at diagnosis regardless of age. (29) In patients with AS-AIH, the prevalence of previously undiagnosed underlying cirrhosis was almost twice as high at 57%. Strictly speaking, these patients have an acute-on-chronic presentation of AIH.…”

Section: Discussionmentioning

confidence: 98%

Early Predictors of Short‐Term Prognosis in Acute and Acute Severe Autoimmune Hepatitis

et al. 2020

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Presentation of autoimmune hepatitis (AIH) can differ from nonacute to acute autoimmune hepatitis (A-AIH) with jaundice and acute severe autoimmune hepatitis (AS-AIH) with jaundice and coagulopathy. The aim of the study was to evaluate the short-term prognosis of different presentations of AIH and the influence of liver function improvement on short-term prognosis. In this single-center retrospective cohort study, AIH patients with repeatedly tested liver function at diagnosis and during at least 1 year of follow-up were included. A-AIH was defined as bilirubin >45 µmol and international normalized ratio (INR) <1.5. AS-AIH was defined as bilirubin level >45 µmol/L and INR ≥1.5. Of the 81 included patients, 17 (21%) presented with A-AIH, and 14 (17%) presented with AS-AIH. After the start of immunosuppressive therapy, bilirubin, albumin, and INR normalized in 70%, 77%, and 69%, respectively, in a median of 2.6 months, 3 months, and 4 weeks, respectively, in patients with A-AIH and AS-AIH. Liver transplantation (LT)-free survival rate was 100% in nonacute AIH, 94% in A-AIH, and 57% in AS-AIH at 12 months after diagnosis. An increase of INR or bilirubin at 2 weeks was the best predictive factor for the need of LT within 12 months with a Youden's index of 0.85. A-AIH was present in 21%, and AS-AIH was present in 17% of AIH patients. In the majority of patients, bilirubin, albumin, and INR normalized in the first months of treatment. Deterioration of liver function after 2 weeks of treatment should lead to rapid evaluation for LT and consideration of second-line medication.

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“…A largesized cross-sectional study reported in the United Kingdoms in 2018 omitted comparative analyses between the probable and de nite AIH patients [27]. Another study prospectively evaluated prognostic factors of AIH using a cohort of 325 patients including both probable and de nite diseases [28]. The study identi ed age at diagnosis as a risk factor for liver-related death or transplant-free survival.…”

Section: Discussionmentioning

confidence: 99%

Prognosis of Probable Autoimmune Hepatitis Patients in a Retrospective Cohort Study

Fujita

Oura

Tadokoro

et al. 2020

Preprint

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Background: Autoimmune hepatitis (AIH) is an idiopathic inflammatory liver disease with genetic susceptibility and unknown environmental triggers, which results in failures of physiologic immunotolerance and destruction of the liver tissues. The gold standard for diagnosis is the International Autoimmune Hepatitis Group (IAIHG) scoring system: the disease is classified as definite or probable according to the scores. However, conventional research on probable AIH has focused on the Caucasian population and there is little data pertaining to the Asian population. Therefore, this study aimed to assess and compare the prognosis of Japanese patients with probable and definite AIH. Methods: Patients with probable and definite AIH diagnosed based on IAIHG scores between 1987 and 2018 were enrolled in this retrospective study. Results: Seventy-two patients with definite AIH and 49 patients with probable AIH were evaluated in the study. Univariate analysis revealed age, fibrosis stage 4, and the fibrosis-4 index were prognostic factors for overall survival. Multivariate analysis indicated that age and liver cirrhosis significantly affected the overall survival. When the cut off albumin-bilirubin score was set appropriately, cirrhosis was differentially diagnosed using albumin-bilirubin score with 100% sensitivity and 70.5% specificity. Classification of probable or definite disease did not alter overall survival with statistical significance. Conclusions: Our findings suggest that probable AIH should be managed as definite AIH is managed in Japanese population. The albumin-bilirubin score helps identify liver cirrhosis and is a prognostic biomarker for overall survival.

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

Cited by 27 publications

References 29 publications

Autoimmune hepatitis: Clinical characteristics and predictors of biochemical response to treatment

Autoimmune hepatitis: Clinical characteristics and predictors of biochemical response to treatment

Early Predictors of Short‐Term Prognosis in Acute and Acute Severe Autoimmune Hepatitis

Prognosis of Probable Autoimmune Hepatitis Patients in a Retrospective Cohort Study

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