[HTML][HTML] The pyrin inflammasome, a leading actor in pediatric autoinflammatory diseases

S La Bella, A Di Ludovico, G Di Donato… - Frontiers in …, 2024 - frontiersin.org
The activation of the pyrin inflammasome represents a highly intriguing mechanism
employed by the innate immune system to effectively counteract pathogenic agents. Despite …

The safety of canakinumab in systemic juvenile idiopathic arthritis and autoinflammatory diseases in pediatric patients: a multicenter study

T Coşkuner, Ş Çağlayan, Ö Akgün… - Expert opinion on …, 2023 - Taylor & Francis
Objective To evaluate the safety of canakinumab using real-world data in patients with
systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID). Research …

[HTML][HTML] Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome?

G Otar Yener, İ Aktaş, C Altıntaş Meşe… - European Journal of …, 2023 - Springer
The primary aim of this study was to document the treatment modalities used in periodic
fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and look for the …

Genetic and clinical features of familial mediterranean fever (FMF) in a homogeneous cohort of patients from South-Eastern Italy

A Di Ciaula, M Iacoviello, L Bonfrate, M Khalil… - European Journal of …, 2023 - Elsevier
Abstract Familial Mediterranean Fever (FMF) is linked with the MEFV gene and is the
commonest among monogenic autoinflammatory diseases, with high prevalence in the …

Colchicine resistance: Associated factors and its effect on health‐related quality of life in patients with familial Mediterranean fever

D Erdem Gürsoy, HH Gezer, N Öz… - International Journal …, 2022 - Wiley Online Library
Aim To determine the factors associated with colchicine resistance and the effect of
colchicine resistance on health‐related quality of life (QoL) in patients with familial …

Not easy-peasy to diagnose: familial Mediterranean fever unaccompanied by fever

SD Arık, GK Kayaalp, V Guliyeva, FG Demirkan… - European Journal of …, 2023 - Springer
Classical attacks of familial Mediterranean fever (FMF) are often accompanied by fever, but
some of the patients have attacks without fever. This study aimed to compare the …

Genotype-Phenotype Associations of Children With Familial Mediterranean Fever in a Cohort Consisting of M694V Mutation and Implications for Colchicine-Resistant …

H Kisaoglu, O Baba, M Kalyoncu - JCR: Journal of Clinical …, 2023 - journals.lww.com
Objectives The aim of this study was to investigate the clinical associations of the second
allele mutations and the effect of genotype and presenting features on colchicine resistance …

[HTML][HTML] IL-1 Inhibitors in the Treatment of Familial Mediterranean Fever: Treatment Indications and Clinical Features in a Large Real-World Cohort

M Yalcin-Mutlu, OC Icacan, F Yildirim… - Journal of Clinical …, 2024 - mdpi.com
Background: The accruing evidence about the efficacy of anti-IL-1 agents in Familial
Mediterranean Fever (FMF) patients led to their widespread off-label use. Therefore …

Cardiovascular manifestations of monogenic periodic fever syndromes

HE Sönmez, Y Bayındır, ED Batu - Clinical Rheumatology, 2023 - Springer
Periodic fever syndromes (PFS) are a group of autoinflammatory diseases characterized by
repeated febrile episodes and systemic inflammation. The most common monogenic …

Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA) …

S Ozdel, T Coşkuner, F Demirkan, R Torun… - Clinical …, 2024 - Springer
Aim The aim of this study was to investigate the frequency and type of FMF-associated
inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF …