Activity

The critical site where these processes occur is the small intestine to which this updated review is dedicated. Understanding its anatomy, its barrier structure and permeability modulation and its microbiota composition is the essential skill to comprehend the complex pathogenesis of several – not only gastroenterological – diseases.Autoimmune Hepatitis (AIH) is a rare autoimmune disease of the liver with many open questions as regards its aetiopathogenesis, natural history and clinical management. The classical picture of AIH is chronic hepatitis with fluctuating elevation of serum transaminases and Immunoglobulin G levels, the presence of circulating autoantibodies and typical histological features. However, atypical presentations do occur and are not well captured by current diagnostic scores, with important consequences in terms of missed diagnoses and delayed treatments. AIH is treated with corticosteroids and immunosuppressive drugs but up to 40% of patients do not achieve full biochemical response and are at risk of progressing to cirrhosis and liver failure. Moreover, standard therapies are associated by significant side-effects which may impair the quality of life of patients living with AIH. However, advances in the understanding of the underlying immunology of AIH is raising the prospect of novel therapies and optimisation of existing therapeutic approaches to reduce side-effect burdens and potentially restore immunological tolerance. In this review we outline the clinical characteristics, aetiopathogenesis and management of AIH and current challenges in the diagnosis and management of AIH and provide evidence underlying the evolution of diagnostic and clinical management protocols.Eosinophilic esophagitis (EoE) is a clinicopathological disease defined by symptoms of esophageal dysfunction and ≥15 eosinophils/HPF after excluding other causes of esophageal eosinophilia. Increasing attention has been paid by clinicians and researchers after its first description in 1978. Many consensuses and guidelines have been issued over the years, as gastroenterologists did not reach an agreement on EoE definition, especially regarding the controversial responsiveness to proton pump inhibitor (PPI) therapy. Of note, recent evidence suggests that the incidence and prevalence of EoE have been increasing through the years many risk factors have been advocated as possible reasons for this, although further studies are needed. In this brief review, we will first cover the history of EoE in the literature, with a focus on its varying definition throughout the years. Then, we will discuss EoE epidemiology, emphasizing potential risk factors explaining its increasing incidence and prevalence. Last, we will deal with the quality of life of adult and pediatric patients with EoE.Dietary elimination therapy has long been an option for patients with eosinophilic esophagitis (EoE) and remains the only therapy targeting the cause of the disease. Different dietary approaches have been described along the last 3 decades, and cumulative evidence has defined the effectiveness and usefulness of each approach. Elemental diets are highly effective to induce EoE remission, but unpractical in the vast majority of patients. Allergy testing-directed food restrictions resulted inefficient to induce remission in a significant proportion of patients (specially adults) and show a low concordance with the dietary causes of EoE. Empiric elimination diets are currently considered the most effective drug-free treatment for patients of all ages with EoE, after providing widely reproducible results. Highly restrictive empiric six-food elimination diets have paved the way to most efficient and less restrictive step-up approaches, which now include four-food and two-food elimination diets. The potential role of milk-elimination, especially in children, should be also considered. Multiple factors including demographics, nutritional status, patient and family lifestyles, social and financial support, and acceptance of repeated endoscopies influence the results of dietary therapy. Dietary therapy in EoE should be patient centered, and the patients and/or their families together with the medical provider should participate in the decision to set up this treatment. This article updates recent knowledge on dietary therapy for EoE and provides guideline to choose the most suitable alternative for patients with EoE, as well as practical tips to achieve the best results in clinical practice.The term IgG4-related autoimmune liver disease (AILD) refers to hepato-biliary manifestations of IgG4-related disease (IgG4-RD) including IgG4-related sclerosing cholangitis and IgG4-related pseudotumors. The association of some forms of autoimmune hepatitis to IgG4-RD remains controversial. Although autoimmune phenomena have not been clearly observed in IgG4-AILD, perturbation of the adaptive immune system and activation of the humoral response represent established pathophysiological hallmarks and potential therapeutic targets. Clinical manifestations of IgG4-AILD are virtually indistinguishable from bile duct cancer or primary sclerosing cholangitis, and are due to mass forming lesions and thickening of the biliary tract that progressively lead to biliary ducts obstruction. There are no current reliable biomarkers for IgG4-AILD and diagnosis should rely on the integration of clinical, serological, radiological, and histological findings. In analogy to most IgG4-RD manifestations, and in contrast to its major mimickers, IgG4-AILD promptly responds to glucocorticoids but frequently relapses, thus requiring long-term maintenance therapy to avoid progressive fibrosclerotic disease and liver cirrhosis. Accumulating evidence on the efficacy of B-cell depletion therapy in patients with systemic IgG4-RD is gradually changing the treatment paradigm of IgG4-AILD and biologics will be increasingly used also for gastroenterological manifestations of IgG4-RD to spare glucocorticoids and traditional immunosuppressive agents. PMA activator cost Looking ahead, identification of reliable biomarkers and of miniinvasive strategies to obtain informative biopsies from the biliary tree represent unavoidable priorities to optimize diagnosis and management of IgG4-AILD.