CONCLUSIONS: Patients with three inherited autoinflammatory syndromes experienced sustained improvements on their HRQoL, work/school, and social life on treatment with canakinumab.

[Figure: see text].

CONCLUSION: Patients with the rheumatic disease most frequently presented to the ED with musculoskeletal complaints and were discharged from the ED. True rheumatological emergencies are rare, but ED physicians should be aware of serious and life-threatening conditions.

CONCLUSION: This systematic literature review characterized the ocular involvement of several AIDs, and the present data may encourage to consider a timely ophthalmological screening program for these rare diseases.

Chromatin 3D structure plays a crucial role in regulation of gene activity. Previous studies have envisioned spatial contact formations between chromatin domains with different epigenetic properties, protein compositions and transcription activity. This leaves specific DNA sequences that affect chromosome interactions. The Drosophila melanogaster polytene chromosomes are involved in non-allelic ectopic pairing. The mutant strain agn^(ts3), a Drosophila model for Williams-Beuren syndrome, has an...

PURPOSE OF REVIEW: Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. Although the gene associated with the disease was identified 24 years ago, we still have to learn about the pathogenesis of its inflammation and the variation in the phenotype. In this review, we discuss some recent findings in FMF, such as changes in our understanding of the genetics, aims to define new criteria, and factors contributing to the disease presentation.

Dynamic early-phase PET images acquired with radiotracers binding to fibrillar amyloid-beta (Aβ) have shown to correlate with [^(18)F]fluorodeoxyglucose (FDG) PET images and provide perfusion-like information. Perfusion information of static PET scans acquired during the first minute after radiotracer injection (FMF, first-minute-frame) is compared to [^(18)F]FDG PET images. FMFs of 60 patients acquired with [^(18)F]florbetapir (FBP), [^(18)F]flutemetamol (FMM), and [^(18)F]florbetaben (FBB) are...

CONCLUSIONS: The present findings show that it may be feasible to withdraw CAN or extend its dose interval in paediatric crFMF patients. Based on the present findings, we think that as the quantity of real-life data increases, standard CAN protocols may be developed.

CONCLUSIONS: Canakinumab is an effective treatment for FMF after failure of anakinra due to any cause.

CONCLUSION: We suggest that FMF plays a role in the etiologic differential diagnosis of cryptogenic cirrhosis.

CONCLUSION: Evaluation of disease severity according to the ISSF in paediatric patients is more sensitive and specific than the Pras and Mor scoring systems.

CONCLUSIONS: Neither FMF nor baseline colchicine therapy, appear to affect the incidence of hospitalization for COVID-19 or the disease course, in terms of severity and therapeutic approach.

CONCLUSION: Dental caries and periodontal disease, which are public health problems, were seen at a high percentage of children with FMF in our study.

CONCLUSIONS: Our data suggest, that the influence of SAA1 genotypic variation on the age of disease onset restricts to FMF patients homozygous for MEFV mutation M694V.

CONCLUSIONS: This is the first study to identify miRNAs that might be associated with a more severe disease form of monogenic autoinflammatory diseases. All these miRNAs were associated with cytokine-mediated pathways and might be used for establishing diagnostic and therapeutic methods.

CONCLUSIONS: Among an international cohort of children with FMF, we did not find a strong effect of gut microbiota composition on disease severity. Other environmental or epigenetic factors may be operative.

CONCLUSIONS: Anakinra and canakinumab seem to be effective in long-term management of FMF patients. Canakinumab had a favourable safety/tolerability profile. Anakinra is also generally safe, but the serious side effects that may be observed in the short and long-term use should be taken into account.

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Systemic autoinflammatory diseases are a heterogeneous family of disorders characterized by a dysregulation of the innate immune system, in which sterile inflammation primarily develops through antigen-independent hyperactivation of immune pathways. In most cases, they have a strong genetic background, with mutations in single genes involved in inflammation. Therefore, they can derive from different pathogenic mechanisms at any level, such as dysregulated inflammasome-mediated production of...

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