Effects of interleukin-1 antagonists on de novo and pre-existing damage in patients with familial Mediterranean fever

Yıldırım, Derya; Ozkiziltas, Burcugul; Vasi, I.; Karadeniz, Hazan; Güler, Aslıhan Avanoğlu; Kardaş, Rıza Can; Küçük, Hamit; Göker, Berna; Öztürk, Mehmet Akif; Tufan, Abdurrahman

doi:10.55563/clinexprheumatol/cbiyem

Clinical and Experimental Rheumatology

2022

DOI: 10.55563/clinexprheumatol/cbiyem

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Effects of interleukin-1 antagonists on de novo and pre-existing damage in patients with familial Mediterranean fever

Derya Yıldırım

Burcugul Ozkiziltas

I. Vasi

et al.

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Old paradigms and new concepts in familial Mediterranean fever (FMF): an update 2023

Ben-Chetrit

2023

Rheumatology

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Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent attacks of fever and polyserositis. Its first description as a new entity was published by Siegal in 1945. Colchicine has been the treatment of choice for this disease since 1972. Significant progress has been made over the years in understanding FMF's clinical features, diagnosis, mode of inheritance, pathogenesis, and therapeutic approach. However, many old paradigms related to FMF have proven inaccurate, leading to the emergence of new concepts that provide more precise insights. The term “FMF” is no longer appropriate as the disease is found beyond the Mediterranean basin. The concept of diagnosis based only upon clinical ground proved to be wrong. The paradigm that MEFV mutations in FMF lead to loss of function of the encoded peptide pyrin, turned out to be a gain of function mutation. Finally, the concept that as a genetic disease FMF should be treated for life, was found to be inaccurate for the subpopulation of the heterozygote patients. Thus, the breakthroughs of identifying the gene associated with the disease (MEFV) and the deciphering of its pathogenesis revolutionized our old paradigms and replaced them with new and more precise insights.

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Old paradigms and new concepts in familial Mediterranean fever (FMF): an update 2023

Ben-Chetrit

2023

Rheumatology

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Effects of interleukin-1 antagonists on de novo and pre-existing damage in patients with familial Mediterranean fever

Cited by 1 publication

References 0 publications

Old paradigms and new concepts in familial Mediterranean fever (FMF): an update 2023

Old paradigms and new concepts in familial Mediterranean fever (FMF): an update 2023

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