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Rheumatology International
Published in: Rheumatology International 1/2018

01-01-2018 | Review Article

Tumor necrosis factor-associated periodic syndrome in adults

Authors: Sharika Gopakumar Menon, Petros Efthimiou

Published in: Rheumatology International | Issue 1/2018

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Abstract

Tumor necrosis factor-associated periodic syndrome is an autoinflammatory disorder classified under hereditary periodic fever syndromes. Mutations in the tumor necrosis factor receptor contribute to tumor necrosis factor-associated periodic syndrome. Decreased shedding of receptors and increased mitochondrial reactive oxygen species production leading to elevated proinflammatory cytokines are documented. Inflammation in various organs is hallmark of tumor necrosis factor-associated periodic syndrome and manifests as spiking fever, abdominal pain, conjunctivitis and polyserositis in adults. The ongoing challenge is to diagnose the disease early in its course to prevent amyloidosis. The treatment options have evolved from use of nonsteroidal anti-inflammatory drugs and corticosteroids to targeted therapy like tumor necrosis factor receptor inhibitors and interleukin-1 blockers. The aim of this review is to give an overview of the pathogenesis, clinical features and the various treatment modalities available for tumor necrosis factor-associated periodic syndrome and aid physicians in recognizing the signs of the disease earlier.
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Metadata
Title
Tumor necrosis factor-associated periodic syndrome in adults
Authors
Sharika Gopakumar Menon
Petros Efthimiou
Publication date
01-01-2018
Publisher
Springer Berlin Heidelberg
Published in
Rheumatology International / Issue 1/2018
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-017-3820-4

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