PERIODIC FEVER SYNDROMES AND OTHER INHERITED AUTOINFLAMMATORY DISEASES

2011 ◽  
pp. 642-660 ◽  
Author(s):  
Karyl Barron ◽  
Balu Athreya ◽  
Daniel Kastner
Keyword(s):  
Periodic Fever ◽  
Autoinflammatory Diseases ◽  
Periodic Fever Syndromes ◽  
Fever Syndromes

scholarly journals Periodic fever syndromes and the autoinflammatory diseases (AIDs)

2020 ◽  
Vol 3 ◽  
pp. 100031
Author(s):  
Achille Marino ◽  
Francesca Tirelli ◽  
Teresa Giani ◽  
Rolando Cimaz
Keyword(s):  
Periodic Fever ◽  
Autoinflammatory Diseases ◽  
Periodic Fever Syndromes ◽  
Fever Syndromes

Autoinflammatory diseases

2013 ◽  
pp. 1410-1417
Author(s):  
Helen J. Lachmann ◽  
Philip N. Hawkins
Keyword(s):  
Innate Immunity ◽  
Inflammatory Disease ◽  
Periodic Fever ◽  
Genetic Diseases ◽  
Autoinflammatory Diseases ◽  
Periodic Fever Syndromes ◽  
Blocking Agents ◽  
Rare Genetic Diseases ◽  
Fever Syndromes

The autoinflammatory diseases are disorders of innate immunity which result in chronic or recurrent multisystem inflammatory disease. This chapter concentrates on the currently known inherited periodic fever syndromes. These rare genetic diseases have resulted in major advances in the understanding of innate immunity in general and the regulation of interleukin (IL)-1 in particular. It now seems clear that autoinflammatory conditions are disorders of the regulation of IL-1 and NFκ‎‎B. The treatment and prognosis of these diseases have been radically improved by the availability of cytokine-blocking agents.

scholarly journals Autoinmunidad y autoinflamación

2019 ◽  
Vol 36 (2) ◽  
pp. 78-84
Author(s):  
Carlos Alberto Cañas Dávila
Keyword(s):  
Periodic Fever ◽  
Autoinflammatory Diseases ◽  
Toll Like Receptor ◽  
Periodic Fever Syndromes ◽  
Revisión Sistemática ◽  
Fever Syndromes

Objetivo: exponer los aspectos que tienen en común o aquellos que diferencien las condiciones autoinmunes y autoinflamatorias, con énfasis en los mecanismos relacionados con la inmunidad innata. Métodos: se realiza una revisión sistemática de la literatura médica expuesta en la base de datos Medline (en lo que respecta a trabajos originales y revisiones de tema de los autores de dichos trabajos), de aspectos de la inmunidad innata y su relación con las enfermedades autoinmunes y autoinflamatorias, utilizando términos “MESH” como “autoimmuny diseases, autoinflammatory diseases, periodic fever syndromes, Toll-like receptor, NOD-like receptor” y otros que fuesen necesarios para lograr el objetivo de la revisión. Se procede luego a la consecución de los artículos completos, a su lectura, complementación con artículos referenciados relevantes, y luego se procede al ordenamiento, clasificación y posterior redacción del texto. Resultados: se estudiaron 254 resúmenes, encontrando que 44 de ellos informaban los tópicos que ayudarán a desarrollar el objetivo de esta revisión. Fue necesario acudir a algunas ayudas extras en libros de inmunología, reumatología, biología molecular y biología celular, para complementar la actualización. Conclusión: apenas se inicia el conocimiento científico de los posibles enlaces directos de la respuesta inmune innata y adaptativa, en lo que respecta a las condiciones autoinmune y autoinflamatoria

scholarly journals MO961KIDNEY TRANSPLANTATION AS A TREATMENT OF CHOICE FOR AA AMYLOIDOSIS DUE TO PERIODIC FEVER SYNDROME

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Ivana Dedinska ◽  
Karol Graňák ◽  
Matej Vnučák
Keyword(s):  
Early Childhood ◽  
Kidney Transplantation ◽  
Clinical Course ◽  
Periodic Fever ◽  
Periodic Fever Syndrome ◽  
Aa Amyloidosis ◽  
Periodic Fever Syndromes ◽  
Post Transplant ◽  
Fever Syndromes ◽  
End Stage

Abstract Background and Aims Renal AA amyloidosis is the most serious complication of periodic fever syndrome, which, inadequate suppression, due to persistent inflammation, leads to nephrotic syndrome and renal failure over several years. In most cases, periodic fever syndromes begin to manifest clinically in early childhood. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment for end-stage chronic kidney disease (CKD) based on AA amyloidosis. Method We present cases of two patients after deceased donor kidney transplantation, who have been diagnosed with adult periodic fever syndrome. Conclusion Periodic fever syndromes are diseases beginning to manifest clinically in early childhood in 60–90% of cases, most often in the case of familial Mediterranean fever around 4 years of age, in the case of CAPS usually only a few months after birth. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation is an effective and safe treatment of end-stage CKD based on AA amyloidosis associated with periodic fever syndrome. Adequate targeted treatment against IL-1 or TNF is important and appears to be safe during the post-transplant period, with regular monitoring of renal function, acute phase inflammatory reactants, and histological findings by protocol graft biopsies. It will be important and necessary to assess the development of diseases in the post-transplant period in the long term.

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