Mistakes in Familial Mediterranean Fever and how to avoid them

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Familial Mediterranean fever (FMF), also called periodic disease, Armenian disease, etc., is a prototypical autoinflammatory disorder where the underlying mechanism is the dysfunction of innate immunity, resulting in unprovoked episodes of inflammation.¹ Although considered rare worldwide, it is prevalent in people of Mediterranean origin; however, one can expect to encounter patients in all parts of the modern world. FMF is a monogenic disease with autosomal recessive inheritance.² Unlike other monogenic disorders, the diagnosis remains largely clinical, and it is important to understand the limitations of genetic testing. Another distinguishing feature is the well-established effectiveness of lifelong monotherapy with colchicine in preventing attacks and complications.³

Mistakes in Familial Mediterranean Fever and how to avoid them

Manik Gemilyan, Gagik Hakobyan

Topics

Primary Care

Published

2025

UEG Podcast Episode

UEG Podcast

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Coeliac disease with David Sanders

David S. Sanders, Pradeep Mundre

Topics

Small Intestine & Nutrition

Published

2026

UEG Podcast Episode

UEG Podcast

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Pancreatic cystic neoplasms with Marco del Chiaro

Marco Del Chiaro, Egle Dieninyte - Misiune

Topics

Pancreas

Published

2025

UEG Podcast Episode

New

UEG Podcast

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Part 1: Nageshwar Reddy - How endoscopy has changed

Duvvur Nageshwar Reddy, Pradeep Mundre

Topics

Endoscopy

Published

2026

UEG Podcast Episode

Journal Podcast

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Episode 6: UEG Journal October Spotlight

Mohsan Subhani, Maria Manuela Estevinho

Topics

Endoscopy Hepatobiliary IBD Pancreas

Published

2025

UEG Podcast Episode

New

UEG Podcast

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EoE with Alex Straumann - Part 2

Egle Dieninyte - Misiune, Alex Straumann

Topics

Oesophagus

Published

2026

UEG Standards and Guidelines

Clinical Practice Guideline

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Summary

Vascular diseases of the liver include portal vein thrombosis (with or without cirrhosis), portosinusoidal vascular disorder, Budd-Chiari syndrome, sinusoidal obstruction syndrome, non-obstructive sinusoidal dilatation and peliosis, splanchnic artery aneurysms, and hepatic arteriovenous fistulas. Except for portal vein thrombosis in cirrhosis, these are all rare conditions. Since the last Clinical Practice Guidelines were issued by the European Association for the Study of the Liver in 2016, much data has been published on the diagnosis and management – medical and interventional – of patients with vascular liver diseases. Based on a thorough review of the relevant literature, recommendations are provided to address key clinical dilemmas. The document emphasises personalised care, considering individual risk factors and clinical presentation. Multidisciplinary management involving hepatologists, haematologists, pathologists, interventional radiologists and surgeons is essential in this area. Our aim is to provide guidance on the management of patients with vascular liver diseases based on the best available evidence.

EASL Clinical Practice Guidelines on vascular diseases of the liver

Publisher

European Association for the Study of the Liver

Guideline

Clinical Practice Guideline

Topics

Hepatobiliary

Citation

Journal of Hepatology; Volume 84, Issue 2, 399-456

Published

2025