Fulminant presentation of autoimmune hepatitis

Mendizábal, Manuel; Marciano, Sebastián; Videla, María Grazia; Anders, Margarita; Zerega, Alina; Balderramo, Domingo; Baña, Matías R. Tisi; Barrabino, Martín; Gil, O.; Mastaï, Ricardo; Yantorno, Silvina; Gadano, Adrián; Silva, Marcelo O.

doi:10.1097/meg.0000000000000353

Cited by 51 publications

(27 citation statements)

References 24 publications

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“…Patients presenting with AIH can benefit from the early use of corticosteroids. Although the risks and benefits of corticosteroid administration in this setting should be weighed carefully, prompt diagnosis and treatment of these patients might preclude LT or death …”

Section: Discussionmentioning

confidence: 99%

Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

et al. 2019

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After the implementation of universal hepatitis A virus vaccination inArgentina, the outcome of pediatric acute liver failure (PALF) remains unknown. We aimed to identify variables associated with the risk of liver transplantation (LT) or death and to determine the causes and short-term outcomes of PALF in Argentina. We retrospectively included 135 patients with PALF listed for LT between 2007 and 2016. Patients with autoimmune hepatitis (AIH), Wilson's disease (WD), or inborn errors of metabolism (IEM) were classified as PALF-chronic liver disease (CLD), and others were classified as "pure" PALF. A logistic regression model was developed to identify factors independently associated with death or need of LT and risk stratification. The most common etiologies were indeterminate (52%), AIH (23%), WD (6%), and IEM (6%). Overall, transplant-free survival was 35%, whereas 50% of the patients underwent LT and 15% died on the waiting list. The 3-month risk of LT or death was significantly higher among patients with pure PALF compared with PALF-CLD (76.5% versus 42.5%; relative risk, 1.8 [1.3-2.5]; P < 0.001), and 3 risk factors were independently associated with worse outcome: international normalized ratio (INR) ≥3.5 (odds ratio [OR], 3.1; 95% confidence interval [CI], 1.3-7.2]), bilirubin ≥17 mg/dL (OR, 4.4; 95% CI, 1.9-10.3]), and pure PALF (OR, 3.8; 95% CI, 1.6-8.9). Patients were identified by the number of risk factors: Patients with 0, 1, or ≥2 risk factors presented a 3-month risk of worse outcome of 17.6%, 36.6%, and 82%, respectively. In conclusion, although lacking external validation, this simple risk-staging model might help stratify patients with different transplant-free survival rates and may contribute to establishing the optimal timing for LT. Liver Transplantation 26 268-275 2020 AASLD.

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Section: Discussionmentioning

confidence: 99%

Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

et al. 2019

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“…Autoimmune hepatitis (AIH) is a complex disease characterized by immune-mediated destruction of hepatic parenchyma, female gender bias, presence of auto-antibodies, hypergammaglobulinaemia, association with other autoimmune conditions, and excellent response to immunosuppressive therapy[ 1 ]. Since its first description by Waldenström in the early 1950’s, AIH was considered to manifest as a chronic liver disease and its fulminant presentation was not commonly reported[ 2 - 4 ]. Over the last decades, it has become apparent that AIH can occur with diverse clinical phenotypes and its classical perception of a chronic inflammatory liver disease that affects mainly young Caucasian women has been expanded[ 5 - 8 ].…”

Section: Introductionmentioning

confidence: 99%

“…Infectious triggers are commonly indicated as being involved in the induction of autoimmune diseases, with Epstein-Barr (EBV) or Cytomegalovirus (CMV) being implicated in several autoimmune liver disorders, such as type I autoimmune hepatitis or primary biliary cholangitis (PBC)[ 9 ]. A remarkable proportion of patients with acute manifestation can develop acute liver failure (ALF), particularly in case of delayed diagnosis and treatment[ 4 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

Potential triggering factors of acute liver failure as a first manifestation of autoimmune hepatitis-a single center experience of 52 adult patients

Buechter¹,

Manka²,

Heinemann³

et al. 2018

WJG

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AIMTo investigate potential triggering factors leading to acute liver failure (ALF) as the initial presentation of autoimmune hepatitis (AIH).METHODSA total of 565 patients treated at our Department between 2005 and 2017 for histologically-proven AIH were retrospectively analyzed. However, 52 patients (9.2%) fulfilled the criteria for ALF defined by the “American Association for the Study of the Liver (AASLD)”. According to this definition, patients with “acute-on-chronic” or “acute-on-cirrhosis” liver failure were excluded. Following parameters with focus on potential triggering factors were evaluated: Patients’ demographics, causation of liver failure, laboratory data (liver enzymes, MELD-score, autoimmune markers, virus serology), liver histology, immunosuppressive regime, and finally, outcome of our patients.RESULTSThe majority of patients with ALF were female (84.6%) and mean age was 43.6 ± 14.9 years. Interestingly, none of the patients with ALF was positive for anti-liver kidney microsomal antibody (LKM). We could identify potential triggering factors in 26/52 (50.0%) of previously healthy patients presenting ALF as their first manifestation of AIH. These were drug-induced ALF (57.7%), virus-induced ALF (30.8%), and preceding surgery in general anesthesia (11.5%), respectively. Unfortunately, 6 out of 52 patients (11.5%) did not survive ALF and 3 patients (5.7%) underwent liver transplantation (LT). Comparing data of survivors and patients with non-recovery following treatment, MELD-score (P < 0.001), age (P < 0.05), creatinine (P < 0.01), and finally, ALT-values (P < 0.05) reached statistical significance.CONCLUSIONDrugs, viral infections, and previous surgery may trigger ALF as the initial presentation of AIH. Advanced age and high MELD-score were associated with lethal outcome.

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“…Prognostic classifications are still not available for this high risk group, yet are key as severity of liver failure may play a role in steroid responsiveness (48). In fact, a study of 40 South American patients with a fulminant AIH revealed that corticosteroid failure was much more likely among those with higher MELD scores and encephalopathy grade 3 or higher (49). Utilization of corticosteroids in an acute severe presentation, preferably at high dose intravenously, requires close observation of clinical improvement or deterioration and infection.…”

Section: Acute Severe Presentationmentioning

confidence: 99%

Management of Difficult Cases of Autoimmune Hepatitis

Lammert

Loy

Oshima

et al. 2016

Curr Gastroenterol Rep

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Autoimmune 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 AASLD and 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.

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Fulminant presentation of autoimmune hepatitis

Cited by 51 publications

References 24 publications

Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

Potential triggering factors of acute liver failure as a first manifestation of autoimmune hepatitis-a single center experience of 52 adult patients

Management of Difficult Cases of Autoimmune Hepatitis

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