Corticosteroid-Responsive Cryptogenic Chronic Hepatitis: Evidence for Seronegative Autoimmune Hepatitis

Gassert, Daniel J.; Garcia, Heriberto; Tanaka, K; Reinus, John F.

doi:10.1007/s10620-006-9665-4

Cited by 78 publications

(59 citation statements)

References 11 publications

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“…[31][32][33] Clinicopathological correlation has shown that NASH and autoimmune hepatitis are leading causes of cryptogenic cirrhosis. [34][35][36][37] Though asymptomatic autoimmune hepatitis is not known, previously unrecognized cirrhosis has been described, especially in patients with acute presentation of autoimmune hepatitis. 38 Positive antinuclear antibody is a feature of autoimmune hepatitis, however, it is a nonspecific finding and frequently seen in patients with NASH and even cryptogenic cirrhosis.…”

Section: Discussionmentioning

confidence: 99%

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Amarapurkar

Amarapurkar²

2015

Journal of Clinical and Experimental Hepatology

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Background: Liver biopsy (LB) has been used as diagnostic modality in liver diseases (LD). Over last two decades, there has been remarkable improvement in understanding of natural history, molecular diagnostics of viral hepatitis, genetic of LD, and also limitations of LB. There is current trend in avoiding LB in the management of various LDs. Aim: To determine utility of LB in clinical practice. Material and Methods: In a prospective study, 2413 patients of LD were followed up, 219 (9%) were acute, and remaining 2194 (90.9%) were chronic LD. Patients were evaluated by biochemical parameters, virological studies, and imaging endoscopy as and when required. LB was performed in 176 (7.2%) patients when no conclusion could be drawn from the noninvasive workup. Patients with platelet count <50,000/cm 2 , ascites, and overt bleeding were excluded. Patients with international normalization ratio (INR) more than 1.5 were not excluded. No prophylactic use of fresh frozen plasma and platelet transfusion was done. There was no major complication related to the procedure. Indications for LB were as follows: cryptogenic LD 38 cases, hepatitis B infection 35, suspected autoimmune hepatitis 30, mass lesion in the liver and lymphoma 29, evaluation of portal hypertension 15, elevated liver enzymes 11, hepatitis C infection 9, and drug-induced LD 4, and miscellaneous 5 cases which were primary biliary cholangitis, primary sclerosing cholangitis, cholestatic LD, sarcoidosis, and amyloidosis. Results: LB changed the diagnosis in 55 (31.2%). These were cryptogenic LD in 24 cases, portal hypertension 15, elevated liver enzymes 11, and 5 others. In remaining, LB confirmed clinical diagnosis and helped in making management decisions. Conclusion: LB was required in 7.2% of patients with chronic LD. In 31.2% cases, LB changed the diagnosis. LB was a safe procedure even in presence of low platelet count and abnormal INR. ( J CLIN EXP HEPATOL 2015;5:314-319)

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Section: Discussionmentioning

confidence: 99%

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Amarapurkar

Amarapurkar²

2015

Journal of Clinical and Experimental Hepatology

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“…To confirm the diagnosis and select further treatment strategies, a liver biopsy and pathological examinations were conducted. Daniel et al (10) described six seronegative AIH patients. These patients, who had no detectable autoantibody markers, were very similar to conventional AIH patients.…”

Section: Discussionmentioning

confidence: 99%

Hashimoto's Thyroiditis Concomitant with Sequential Autoimmune Hepatitis, Chorea and Polyserositis: A New Entity of Autoimmune Polyendocrine Syndrome?

Qiu

Pan

et al. 2013

Intern. Med.

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We herein report a case of Hashimoto's thyroiditis (HT) with sequential autoimmune hepatitis (AIH), chorea and polyserositis. The patient was a 24-year-old man who underwent subtotal thyroidectomy due to compression symptoms caused by goiter and was diagnosed with HT postoperatively based on pathological examinations two years previously. He had exhibited liver dysfunction and intermittent chorea since 2008. His liver function and polyserositis improved remarkably following the administration of ursodeoxycholic acid (UDCA) and methylprednisolone. This is a very rare case that can be classified as autoimmune polyglandular syndrome (APS) type 3. Early and adequate UDCA and glucocorticoid treatment may lead to a favorable prognosis.

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“…Only a small proportion of AIH patients do not have detectable autoantibody seropositivity. In adults, the rare seronegative patients respond to immunosuppression like their seropositive counterparts [19] . The prevalence and clinical characteristics of seronegative AIH remain to be defined in children [1] .…”

Section: Diagnosismentioning

confidence: 99%

Paediatric Autoimmune Liver Disease

Liberal¹,

Vergani²,

Mieli‐Vergani³

2015

Dig Dis

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In paediatrics, there are 2 liver disorders in which liver damage most likely stems from an autoimmune attack: ‘classical' autoimmune hepatitis (AIH) and the AIH/sclerosing cholangitis overlap syndrome (also known as autoimmune sclerosing cholangitis, ASC). The presentation of childhood autoimmune liver disease (AILD) is non-specific and can mimic most other liver disorders. AIH is exquisitely responsive to immunosuppressive treatment, which should be instituted promptly to prevent rapid deterioration and promote remission and long-term survival. Difficult-to-treat or non-responsive patients should be treated with mycophenolate mofetil; if this fails then calcineurin inhibitors can be tried. Persistent failure to respond or lack of adherence to treatment result in end-stage liver disease. These patients, and those with fulminant liver failure at diagnosis, will require liver transplantation. ASC responds to the same immunosuppressive treatment used for AIH when treatment is initiated early. Abnormal liver function tests often resolve within a few months of treatment, although medium- to long-term prognosis is worse than that of AIH because bile duct disease continues to progress despite treatment in approximately 50% of patients. Ursodeoxycholic acid is usually added to conventional treatment regimen in ASC, but whether this actually helps arrest the progression of bile duct disease remains to be established. The pathogenesis of paediatric-onset AILD is not fully understood, although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack. Liver damage is thought to be mediated primarily by CD4^pos T-cells. While Th1 effector cells are associated with hepatocyte damage in both AIH and ASC, Th17 immune responses predominate in the latter where they correlate with biochemical indices of cholestasis, indicating that IL-17 is involved in the bile duct damage characteristic of this condition. Since a substantial difference between these 2 pathologies is the frequent association of ASC with inflammatory bowel disease, it can be speculated that lymphocytes of intestinal origin are present in patients with a diagnosis of ASC rather than in those with AIH. Animal models faithfully representing the human conditions are needed to unravel the contribution of innate and adaptive, effector and regulatory immune responses. A deeper understanding of the pathogenesis of AILD is likely to contribute to the development of novel treatments, such as the adoptive transfer of autologous expanded antigen-specific regulatory T-cells, which ultimately aim at restoring tolerance to liver-derived antigens.

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Corticosteroid-Responsive Cryptogenic Chronic Hepatitis: Evidence for Seronegative Autoimmune Hepatitis

Cited by 78 publications

References 11 publications

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Hashimoto's Thyroiditis Concomitant with Sequential Autoimmune Hepatitis, Chorea and Polyserositis: A New Entity of Autoimmune Polyendocrine Syndrome?

Paediatric Autoimmune Liver Disease

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