Autoimmune sclerosing cholangitis: Evidence and open questions

Beretta‐Piccoli, Benedetta Terziroli; Vergani, Diego; Mieli‐Vergani, Giorgina

doi:10.1016/j.jaut.2018.10.008

Cited by 29 publications

(16 citation statements)

References 71 publications

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“… 31 Pediatric cases of PSC often have positive autoantibodies and significant inflammatory activity resembling autoimmune hepatitis, and, thus, it is sometimes referred to as autoimmune sclerosing cholangitis. 32 Serum IgG4 concentrations are elevated in 10% to 15% of otherwise typical adult PSC, 33 , 34 and this ratio increases up to 40% in pediatric PSC.…”

Section: Controversymentioning

confidence: 97%

Type 2 Autoimmune Pancreatitis: Consensus and Controversies

Zen

2022

Gut and Liver

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Autoimmune pancreatitis (AIP) has attracted much attention in the last two decades, and due to the diagnostic value of immunoglobulin G4 (IgG4), the number of cases diagnosed in clinical practice has markedly increased. However, in contrast to prototypic IgG4-related type 1 AIP, a minor subtype of AIP, referred to as type 2 AIP, is less widely known and has thus not yet been characterized in detail. Type 2 AIP is unrelated to IgG4 and is a completely distinct entity from type 1 AIP. One confusing factor is that the two types of AIP share patterns of clinical presentation (e.g., acute pancreatitis and painless jaundice) and imaging abnormalities (e.g., diffuse or segmental enlargement). Since there are currently no established serum markers, the diagnosis of type 2 AIP is highly challenging and requires the tissue confirmation of neutrophilic injury to the pancreatic ducts, a finding designated as a granulocytic epithelial lesion. Approximately one-third of cases are associated with inflammatory bowel disease, particularly ulcerative colitis; however, the pathological relationship between these two conditions has not yet been clarified. Unanswered questions relate to its pathophysiology, the potential development of a similar granulocytic injury in other organs, and the characteristics of pediatric cases. This review summarizes consensus and controversies surrounding type 2 AIP, with the aim of increasing awareness and highlighting the unmet needs of this underrecognized condition.

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Section: Controversymentioning

confidence: 97%

Type 2 Autoimmune Pancreatitis: Consensus and Controversies

Zen

2022

Gut and Liver

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“…Çocuklarda SK prevelansı 1,5/100.000 olarak bildirilmiştir (7) . Genetik yatkınlık değerlendirildiğinde OSK'in HLA DRB1 * 1301 ile ilişkili olduğu gösterilmiştir (11) . Tip 1 OİH'de olduğu gibi karın ağrısı, kilo kaybı ve aralıklı sarılık OSK'de sık görülen bulgulardır.…”

Section: Sklerozan Kolanjit Ve Overlap Sendromuunclassified

Autoimmune Hepatitis in Children

Aksoy¹,

Appak²,

Baran³

2021

TERH

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Autoimmune hepatitis is characterized by inflammation in liver histology, circulating autoantibodies, and increased levels of immunglobulin G, in the absence of a known etiology. There are different clinical types described in children. Because of the presentation of autoimmune hepatitis with different clinical findings, establishment of diagnosis may be difficult. In the last two decades, a a 6-fold increase in the incidence of autoimmune hepatitis has been reported thanks to update of diagnostic approaches and the increase in technical possibilities. Autoimmune hepatitis should be considered in the differential diagnosis of any liver disease not caused by viral and metabolic factors. Its association with other clinical conditions such as autoimmune thyroiditis, vitiligo, collagen and connective tissue disorders is already known. Although autoimmune hepatitis is a treatable disorder, it may cause cirrhosis and fulminant hepatitis that need liver transplantation without treatment. In this review, we aimed to present the clinical findings, diagnosis and treatment methods of autoimmune hepatittis in children in the light of current literature.

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“…In pediatria, è definita una entità clinica denominata colangite sclerosante autoimmune, che rappresenta una sovrapposizione di AIH con colangite sclerosante, e colpisce la metà dei bambini con AIH (prevalentemente di tipo 1) [17].…”

Section: Caratteristiche Cliniche E Biochimicheunclassified

Le malattie autoimmuni di fegato nell’adulto

Beretta‐Piccoli

Gottardi

Vergani

et al. 2021

Schweiz. Gastroenterol.

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RiassuntoL’epatite autoimmune è una infiammazione cronica del fegato che colpisce tutte le età, caratterizzata da transaminasi e immunoglobuline G elevate, presenza di autoanticorpi, epatite dell’interfaccia alla biopsia epatica, e ottima risposta alla terapia con steroidi. Se non trattata, ha una sopravvivenza a 5 anni del 50 %.La colangite biliare primitiva è una patologia cronica colestatica autoimmune del fegato che colpisce i medi e piccoli dotti biliari, caratterizzata da preponderanza femminile, e positività dell’anticorpo anti-mitocondrio. La sopravvivenza media della malattia non trattata è 9‑10 anni. La terapia di scelta è l’acido ursodesossicolico, che ha un forte impatto sulla storia naturale della malattia.La colangite sclerosante primitiva è la più rara e la più grave delle malattie autoimmuni di fegato. Si caratterizza da forte associazione alle malattie infiammatorie intestinali. I pazienti con PSC hanno un rischio elevato di colangiocarcinoma. Non esistono terapie medicamentose efficaci, e la malattia richiede una presa a carico specialistica multidisciplinare.Questo articolo offre una panoramica per il clinico delle tre patologie nell’adulto.

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Autoimmune sclerosing cholangitis: Evidence and open questions

Cited by 29 publications

References 71 publications

Type 2 Autoimmune Pancreatitis: Consensus and Controversies

Type 2 Autoimmune Pancreatitis: Consensus and Controversies

Autoimmune Hepatitis in Children

Le malattie autoimmuni di fegato nell’adulto

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