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Current Hepatology Reports

Erschienen in:

19.05.2020 | Autoimmune, Cholestatic, and Biliary Diseases (S Gordon and CL Bowlus, Section Editors)

PSC and Overlap Syndromes

verfasst von: Nathalie Pena Polanco, Claudia Cottone, Kalyan Ram Bhamidimarri

Erschienen in: Current Hepatology Reports | Ausgabe 2/2020

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Abstract

Purpose of Review

Overlap syndromes show concomitant or sequential findings of two or more autoimmune liver diseases. Primary sclerosing cholangitis (PSC) is rare and PSC-overlap syndromes are much rarer. There is limited data in PSC-overlap syndromes which pose significant diagnostic and therapeutic challenges for this condition. We summarized the published data in this review to update the management strategies in PSC-overlap syndrome.

Recent Findings

PSC incidence is 0.77–1 per 100,000 person/years, of which 2–10% are reported to have PSC-autoimmune hepatitis (AIH) overlap syndrome. The incidence of PSC-primary biliary cholangitis (PBC) is extremely rare. Inflammatory bowel disease (IBD) is more common in classic PSC than in PSC-AIH patients (80% vs. 63%) and unusual in PSC-PBC. PSC can present with a more hepatocellular form of injury in children, also called autoimmune sclerosing cholangitis (ASC) rather than the typical classic cholestatic presentation in the adults. The presence of overlap syndrome can be diagnosed concomitantly or sequentially during the disease course in an individual. Liver fibrosis progression in PSC-AIH overlap syndrome is similar to classic PSC but hepatobiliary malignancy is reportedly infrequent. Diagnostic scoring criteria are not recommended and clinical diagnosis relies primarily on liver biochemistry, cholangiography and liver biopsy. Ursodeoxycolic acid and immunosuppressive therapy is beneficial in some patients with PSC-overlap syndrome. The biochemical response to pharmacotherapy is higher in PSC-overlap syndrome when compared to classic PSC but lower than that observed in classic AIH.

Summary

PSC overlap syndromes are poorly understood. Current evidence is based on data compiled from case reports and series. This review provides an updated summary that could assist in the clinical management of patients with this complex disorder.

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Titel: PSC and Overlap Syndromes
verfasst von: Nathalie Pena Polanco
Claudia Cottone
Kalyan Ram Bhamidimarri
Publikationsdatum: 19.05.2020
Verlag: Springer US
Erschienen in: Current Hepatology Reports / Ausgabe 2/2020
Elektronische ISSN: 2195-9595
DOI: https://doi.org/10.1007/s11901-020-00518-w

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Wenn unter einer medikamentösen Hochdrucktherapie der diastolische Blutdruck in den Keller geht, steigt das Risiko für schwere kardiovaskuläre Ereignisse: Darauf deutet eine Sekundäranalyse der SPRINT-Studie hin.

Bei schweren Reaktionen auf Insektenstiche empfiehlt sich eine spezifische Immuntherapie

25.04.2024 Allergien und Intoleranzreaktionen Nachrichten

Insektenstiche sind bei Erwachsenen die häufigsten Auslöser einer Anaphylaxie. Einen wirksamen Schutz vor schweren anaphylaktischen Reaktionen bietet die allergenspezifische Immuntherapie. Jedoch kommt sie noch viel zu selten zum Einsatz.

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

25.04.2024 Hypertonie Nachrichten

Beginnen ältere Männer im Pflegeheim eine Antihypertensiva-Therapie, dann ist die Frakturrate in den folgenden 30 Tagen mehr als verdoppelt. Besonders häufig stürzen Demenzkranke und Männer, die erstmals Blutdrucksenker nehmen. Dafür spricht eine Analyse unter US-Veteranen.

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