IBG RareBoost Seminar – Prof. Ahmet Gül “Familial Mediterranean Fever and Beyond: The Spectrum of Pyrin-Associated Autoinflammatory Disorders”
On June 27th at 11:00 am, Prof. Ahmet Gül will give a seminar titled “Familial Mediterranean Fever and Beyond: The Spectrum of Pyrin-Associated Autoinflammatory Disorders” at IBG Aziz Sancar Auditorium.
Prof. Gül is an expert in immunology and rheumatology, with a long-standing academic career at Istanbul University. His research focuses on the immunopathogenesis and genetics of inflammatory rheumatic disorders, especially Behçet’s disease, familial Mediterranean fever (FMF), and other monogenic autoinflammatory disorders.
Prof. Gül has authored over 200 publications and serves on the editorial boards of leading rheumatology journals. He is also the President of the Behçet’s Disease Society of Turkey and President-Elect of the International Society for Behçet’s Disease.
You may join the seminar in person at IBG Aziz Sancar Auditorium or online by signing up via the following link.
We welcome your participation!
Seminar Abstract
Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disorder, predominantly affecting populations in the Eastern Mediterranean. Although classified as a ‘rare disease’; its prevalence exceeds the standard threshold for rarity in certain regions of Israel, Armenia, and Turkey. In 1997, pathogenic variants in exon 10 of the MEFV gene—encoding the pyrin protein—were identified as the genetic cause of FMF. Pyrin is a cytosolic sensor protein that forms part of the inflammasome complex, leading to the activation of caspase-1 and the proteolytic maturation of the proinflammatory cytokines IL-1β and IL-18, which drive the inflammatory episodes seen in FMF.
Clinically, FMF is characterized by recurrent, self-limited episodes of peritonitis, pleuritis, arthritis, and erysipelas-like erythema, typically lasting 12 to 72 hours. In addition to the classic exon 10 mutations, variants in other exons of the MEFV gene have been associated with different phenotypes and prognostic features. The clinical spectrum of pyrin-associated autoinflammatory diseases is expanding, and deeper insights into pyrin-inflammasome signaling are expected to inform both pathogenesis and therapeutic strategies.