scholarly journals Etiopathogenesis and Diagnostic Strategies in Autoimmune Hepatitis

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1418
Author(s):  
Weronika Domerecka ◽  
Anna Kowalska-Kępczyńska ◽  
Agata Michalak ◽  
Iwona Homa-Mlak ◽  
Radosław Mlak ◽  
...  
Keyword(s):  
Liver Failure ◽  
Autoimmune Hepatitis ◽  
Molecular Mimicry ◽  
Disease Onset ◽  
Disease Process ◽  
Elevated Concentration ◽  
Diagnostic Strategies ◽  
Severe Acute Hepatitis ◽  
Circulating Autoantibodies ◽  
End Stage

Autoimmune hepatitis (AIH) is a chronic liver disease with the incidence of 10 to 17 per 100,000 people in Europe. It affects people of any age, but most often occurs in the 40–60 age group. The clinical picture is varied, from asymptomatic to severe acute hepatitis or liver failure. The disease onset is probably associated with the impaired function of T lymphocytes, the development of molecular mimicry, intestinal dysbiosis, or infiltration with low density neutrophils, which, alongside autoantibodies (i.e., ANA, ASMA), implicate the formation of neutrophil extracellular traps (NETs), as a component of the disease process, and mediate the inappropriate immune response. AIH is characterized with an increased activity of aminotransferases, elevated concentration of serum immunoglobulin G, the presence of circulating autoantibodies and liver inflammation. The result of the histological examination of the liver and the presence of autoantibodies, although not pathognomonic, still remain a distinguishing feature. The diagnosis of AIH determines lifelong treatment in most patients. The treatment is implemented to prevent the development of cirrhosis and end-stage liver failure. This work focuses mainly on the etiopathogenesis and diagnosis of AIH.

Autoimmune hepatitis

2020 ◽  
pp. 3119-3127
Author(s):  
G.J. Webb ◽  
Gideon M. Hirschfield
Keyword(s):  
Autoimmune Hepatitis ◽  
Clinical Features ◽  
Elevated Serum ◽  
Clinical Signs ◽  
Decompensated Cirrhosis ◽  
Primary Biliary Cholangitis ◽  
Laboratory Findings ◽  
Circulating Autoantibodies ◽  
End Stage ◽  
Liver Tests

Autoimmune hepatitis is an idiopathic inflammation of the liver attributed to immune responses against self-antigens presumed to be of hepatocyte origin. It is typically a relapsing and remitting corticosteroid-responsive condition associated with hepatitic serum liver tests, elevated gammaglobulins, and positive immune serology. Histological features are not specific but often include expanded portal tracts with a lymphoplasmacytic infiltrate. Epidemiology: predominantly affects women, may occur throughout life, has some heritable component, and 60% of patients have other autoimmune diseases. Clinical features: many patients are asymptomatic and identified through investigation of abnormal serum liver tests. Presentation may be with anorexia, nausea, hepatic discomfort, and jaundice, but others may have nonspecific malaise or extrahepatic manifestations such as arthralgia, arthritis, or fever. Clinical signs vary greatly, ranging from none to jaundice and tender hepatomegaly to fulminant hepatic failure. One-third of patients present as cirrhotic. Diagnosis: characteristic laboratory findings include elevated serum transaminase activities, hypergammaglobulinaemia (as IgG), and circulating autoantibodies (e.g. antismooth muscle antibodies, anti-liver–kidney microsomal antibodies, and antinuclear antibodies). Diagnosis depends on the combination of clinical features and biochemical, immunological, and liver biopsy abnormalities, with exclusion of viral and other aetiologies. There may be overlap features with other autoimmune liver diseases (primary sclerosing cholangitis or primary biliary cholangitis). Treatment and prognosis: the condition tends to progress to hepatic fibrosis and cirrhosis. Most cases should be treated with an immunosuppressive regimen, typically prednisolone with azathioprine in the first instance, and most require long-term immunosuppression. Crude 10-year survival rate is 65% for those presenting with cirrhosis and greater than 95% for those presenting without. End-stage decompensated cirrhosis and acute nonresponsive autoimmune hepatitis with liver failure can be indications for liver transplantation.

scholarly journals Lactic Acidosis in a Congenital Bone Marrow Failure Syndrome

2021 ◽  
pp. 1-4
Author(s):  
Fatima Farid Mir ◽  
Anjan Madasu ◽  
Hani Humad ◽  
Asim Noor Rana
Keyword(s):  
Bone Marrow ◽  
Mitochondrial Dna ◽  
Metabolic Acidosis ◽  
Lactic Acidosis ◽  
Liver Failure ◽  
Bone Marrow Failure ◽  
Disease Course ◽  
Bone Marrow Failure Syndrome ◽  
Severe Lactic Acidosis ◽  
End Stage

Fifteen-month-old male child, known to have a congenital bone marrow failure syndrome, presented in a state of shock with severe lactic acidosis following a brief episode of vomiting. Hospital stay was complicated by recurrent bouts of metabolic acidosis and progressive hepatic failure. Blood mitochondrial DNA sequencing revealed a large heteroplasmic 4,977 bp mitochondrial deletion (approximately 40% of all mitochondrial copies) suggestive of Pearson marrow-pancreas syndrome. By virtue of natural disease course, within a month of admission child succumbed to end-stage liver failure with multi-organ failure and died.

scholarly journals Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis

2017 ◽  
Vol 45 (3) ◽  
pp. 248-256 ◽  
Author(s):  
Fernanda Payan Schober ◽  
Meghan A. Jobson ◽  
Caroline J. Poulton ◽  
Harsharan K. Singh ◽  
Volker Nickeleit ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Disease Process ◽  
Signs And Symptoms ◽  
Patient Characteristics ◽  
Underlying Disease ◽  
Fibrillary Glomerulonephritis ◽  
Black Patients ◽  
End Stage ◽  
20 Nm ◽  
The University

Background: Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described. Methods: The University of North Carolina (UNC) Nephropathology Database was used to retrospectively identify patients diagnosed with fibrillary glomerulonephritis between 1985 and 2015. Of these patients, those treated at UNC were selected. Their demographic and clinical characteristics - including signs and symptoms, comorbidities, laboratory values, treatments and outcomes - were compared with those of patients described earlier. Results: Among the 287 patients identified, 42 were treated at the UNC Kidney Center. When compared to earlier cohorts, a higher frequency of black race, hepatitis C virus (HCV) infection and use of hemodialysis were noted in both black and HCV-positive patients. Autoimmune diseases, infections and malignancies were frequently observed, present in over half of all cases. Conclusion: According to this study, fibrillary glomerulonephritis represents a secondary glomerular disease process (associated with autoimmune disease, infection or malignancy) in many cases and hence screening is essential. As the screening for comorbidities increased over time, more underlying causes were identified. We noted a high frequency of HCV among black patients, suggesting a possible causative association. Treatment of underlying disease is essential for patients for the best outcome.

Sign in / Sign up

Export Citation Format

Share Document