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Item Autoimmune Hepatitis in the Elderly: Diagnosis and Pharmacologic Management(Springer, 2018-07) Rizvi, Syed; Gawrieh, Samer; Medicine, School of MedicineAutoimmune hepatitis (AIH) may present as acute or chronic hepatitis in the elderly. Advanced hepatic fibrosis and cirrhosis are common on first presentation in this population. In this review, we discuss the presentation, approach to diagnosis and management of AIH in the elderly. As polypharmacy is common in the elderly, careful medication use history is essential for detecting drug-induced AIH-like hepatitis. Steroid-sparing or minimizing therapeutic regimens are preferred to treat AIH in the elderly. For the purpose of induction, budesonide or lower dose prednisone in combination with azathioprine (AZA) regimens are preferred over high-dose prednisone monotherapy due to the higher risk of side effects of the later in the elderly. The goal of maintenance therapy should be to achieve full biochemical and histologic remission. Bone density monitoring and interventions to prevent steroid-related bone disease should be implemented throughout the course of the disease. Liver transplantation should be considered in the elderly patient with liver failure or early hepatocellular carcinoma if there are no significant comorbidities or compromise in functional status.Item Behaviors, symptoms, and outcomes of North American patients with autoimmune hepatitis during the COVID-19 pandemic(BMJ, 2021-12) Vuppalanchi, Vahin; Gelow, Kayla; Vuppalanchi, Raj; Lammert, Craig; Green, Kelsey; Medicine, School of MedicineThe management of patients with autoimmune hepatitis (AIH) in the era of SARS-CoV-2 is challenging given minimal published clinical data. We used a large cohort of patients with AIH across the USA to investigate the differences in known risk factors for severe SARS-CoV-2 and AIH characteristics among patients who experienced symptoms consistent with COVID-19 illness versus those who did not. Additionally, we explored the effect of living through the SARS-CoV-2 pandemic on the extrahepatic symptoms and behaviors of patients with AIH. An invitation to complete a COVID-19-specific questionnaire was publicized in well-established social media cohorts of patients with AIH. Eligibility criteria were age ≥18 years, US residency, and an AIH diagnosis by a physician. A total of 420 individuals were eligible for the study. Symptoms consistent with COVID-19 were reported in 11% (n=48) with 3 patients requiring hospitalizations. Body mass index (BMI) >40 kg/m2 (23% vs 10%, p=0.01) and exposure to house (33% vs 3%, p=0.0001) or work (38% vs 17%, p=0.02) contacts with COVID-19 were factors found higher in those with symptoms. Cirrhosis or steroid use or immunosuppression was not significantly different between symptomatic and non-symptomatic groups. Worsening fatigue (45% vs 30%, p=0.06), anxiety (89% vs 70%, p=0.08), and itch (40% vs 18%, p=0.03) were more common among those reporting COVID-19 symptoms compared with those without. BMI >40 kg/m2 and exposure to contacts with COVID-19 illness but not cirrhosis or immunosuppression were associated with increased risk of COVID-19 illness in patients with AIH.Item Consensus Recommendations for Histological Criteria of Autoimmune Hepatitis from the International AIH Pathology Group(Wiley, 2022-05) Lohse, Ansgar W.; Sebode, Marcial; Bhathal, Prithi S.; Clouston, Andrew D.; Dienes, Hans P.; Jain, Dhanpat; Gouw, Annette S. H.; Guindi, Maha; Kakar, Sanjay; Kleiner, David E.; Krech, Till; Lackner, Carolin; Longerich, Thomas; Saxena, Romil; Terracciano, Luigi; Washington, Kay; Weidemann, Sören; Hübscher, Stefan G.; Tiniakos, Dina; Pathology and Laboratory Medicine, School of MedicineBackground & Aims Diagnostic histological criteria for autoimmune hepatitis (AIH) have not been clearly established. Previously published criteria focused mainly on chronic AIH, in which inflammatory changes mainly occur in portal/periportal regions and may not be applicable to acute presentation of AIH, in which inflammatory changes are typically predominantly lobular in location. International consensus criteria for the diagnosis and assessment of disease severity in both acute and chronic AIH are thus urgently needed. Methods Seventeen expert liver pathologists convened at an international workshop and subsequently used a modified Delphi panel approach to establish consensus criteria for the histopathological diagnosis of AIH. Results The consensus view is that liver biopsy should remain standard for diagnosing AIH. AIH is considered likely, if there is a predominantly portal lymphoplasmacytic hepatitis with more than mild interface activity and/or more than mild lobular hepatitis in the absence of histological features suggestive of another liver disease. AIH is also considered likely if there is predominantly lobular hepatitis with or without centrilobular necroinflammation and at least one of the following features: portal lymphoplasmacytic hepatitis, interface hepatitis or portal-based fibrosis, in the absence of histological features suggestive of another liver disease. Emperipolesis and hepatocellular rosettes are not regarded as being specific for AIH. Conclusions The criteria proposed in this consensus statement provide a uniform approach to the histological diagnosis of AIH, which is relevant for patients with an acute as well as a chronic presentation and to more accurately reflect the current understanding of liver pathology in AIH.Item Hepatic steatosis is highly prevalent but is not correlated with stiffness in autoimmune hepatitis(Wolters Kluwer, 2020-10-16) Chalasani, Sai; Mathur, Karan; Shammas, Nicole; Orman, Eric; Vuppalanchi, Raj; Lammert, Craig; Medicine, School of MedicineThe prevalence and impact of hepatic steatosis among patients with autoimmune hepatitis (AIH) is not well described. We conducted a cross-sectional study to determine the prevalence of hepatic steatosis in AIH patients and examined its relationship with hepatic fibrosis using vibration controlled transient elastography. Liver stiffness measurement (LSM), controlled attenuation parameter (CAP), gender, current age, and body mass index (BMI) were collected from 277 AIH patients. Hepatic steatosis was defined as CAP >263 db/m. The study participants were mostly female (82%) with an average age of 49 years and BMI 29.7 kg/m2. Mean LSM was 12.5 (standard deviation 13.5) kPa and CAP was 244 (standard deviation 63) db/m. The prevalence of coexisting steatosis was 33.2%, and steatosis did not correlate with LSM (r = 0.05, P = .46). In this study, only gender (females with 31% lower LSM on average compared to males, P = .001) and BMI (each unit increase of BMI resulted in a 1.48% increase on average LSM, P = .01) correlated with LSM. Male gender had significant association with increased LSM, after controlling for age, BMI, and CAP (P = .001). This exploratory study using noninvasive vibration controlled transient elastography revealed hepatic steatosis is highly prevalent in patients with AIH but not associated with liver fibrosis.Item Investigation Gone Viral: Application of the Social Mediasphere in Research(AGA Institute, 2015-10) Lammert, Craig; Comerford, Megan; Love, Joshua; Bailey, James Robert; Department of Medicine, IU School of MedicineItem Management of Difficult Cases of Autoimmune Hepatitis(Springer, 2016-02) Lammert, Craig; Loy, Veronica M.; Oshima, Kiyoko; Gawrieh, Samer; Department of Medicine, IU School of MedicineAutoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. Current American Association for the Study of Liver Diseases (AASLD) and European Association for the Study of Liver (EASL) guidelines recommend corticosteroids alone or in combination with azathioprine as first-line treatment strategies. However, a significant proportion of patients may not be able to tolerate or achieve complete biochemical response with these options. In this article, we discuss approaches to these patients and other challenging AIH patient groups such as the asymptomatic, pregnant, elderly, and liver transplant recipients.Item Outcome of COVID-19 in Patients with Autoimmune Hepatitis: an International Multi-Centre Study(Wiley, 2021) Efe, Cumali; Dhanasekaran, Renumathy; Lammert, Craig; Ebi, Berat; Higuera‐de la Tijera, Fatima; Aloman, Costica; Rıza Calışkan, Ali; Peralta, Mirta; Gerussi, Alessio; Massoumi, Hatef; Catana, Andreea M.; Torgutalp, Murat; Purnak, Tugrul; Rigamonti, Cristina; Gomez Aldana, Andres Jose; Khakoo, Nidah; Kacmaz, Hüseyin; Nazal, Leyla; Frager, Shalom; Demir, Nurhan; Irak, Kader; Ellik, Zeynep Melekoğlu; Balaban, Yasemin; Atay, Kadri; Eren, Fatih; Cristoferi, Laura; Batıbay, Ersin; Urzua, Álvaro; Snijders, Romee; Kıyıcı, Murat; Akyıldız, Murat; Ekin, Nazım; Carr, Rotonya M; Harputoğlu, Murat; Hatemi, Ibrahim; Mendizabal, Manuel; Silva, Marcelo; Idilman, Ramazan; Silveira, Marina; Drenth, Joost P.H.; Assis, David N.; Björnsson, Einar; Boyer, James L.; Invernizzi, Pietro; Levy, Chyntia; Schiano, Thomas D.; Ridruejo, Ezequiel; Wahlin, Staffan; Medicine, School of MedicineBackground Data regarding outcome of Coronavirus disease 2019 (COVID‐19) in patients with autoimmune hepatitis (AIH) are lacking. Patients and methods We performed a retrospective study on AIH patients with COVID‐19 from 34 centres in Europe and the Americas. We analyzed factors associated with severe COVID‐19 outcomes defined as the need for mechanical ventilation, intensive care admission, and/or death. The outcomes of patients with AIH were compared to a propensity‐score matched cohort of non‐AIH patients with chronic liver diseases (CLD) and COVID‐19. The frequency and clinical significance of new‐onset liver injury (alanine aminotransferase>2xupper limit of normal) during COVID‐19 was also evaluated. Results We included 110 AIH patients (80%,female) with a median age of 49 (range:18–85) years at COVID‐19 diagnosis. New‐onset liver injury was observed in 37.1% (33/89) of the patients. Use of antivirals was associated with liver injury (p=0.041; odds ratio (OR) 3.36[1.05‐10.78]) while continued immunosuppression during COVID‐19 was associated with a lower rate of liver injury (p=0.009; OR 0.26[0.09‐0.71]). The rates of severe COVID‐19 (15.5% vs 20.2% p=0.231) and all‐cause mortality (10% vs 11.5%; p=0.852) were not different between AIH and non‐AIH CLD. Cirrhosis was an independent predictor of severe COVID‐19 in patients with AIH (p<0.001; OR 17.46[4.22‐72.13]). Continuation of immunosuppression or presence of liver injury during COVID‐19 was not associated with severe COVID‐19. Conclusions This international, multi‐center study reveals that patients with AIH were not at risk for worse outcomes with COVID‐19 than other causes of CLD. Cirrhosis was the strongest predictor for severe COVID‐19 in AIH patients. Maintenance of immunosuppression during COVID‐19 was not associated with increased risk for severe COVID‐19, but did lower the risk for new‐onset liver injury during COVID‐19.