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Tumor necrosis factor-associated periodic syndrome in adults

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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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Authors and Affiliations

  1. Rheumatology, New York Presbyterian Hospital-Brooklyn Methodist Hospital, Brooklyn, NY, USA

    Sharika Gopakumar Menon

  2. NYU Langone Medical Center, New York, NY, USA

    Petros Efthimiou

  3. Rheumatology Division, New York University School of Medicine, 501, 1st Avenue, New york, NY, 10016, USA

    Petros Efthimiou

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  1. Sharika Gopakumar Menon
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Sharika Gopakumar Menon and Petros Efthimiou declare that they have no conflict of interest.

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Menon, S.G., Efthimiou, P. Tumor necrosis factor-associated periodic syndrome in adults. Rheumatol Int 38, 3–11 (2018). https://doi.org/10.1007/s00296-017-3820-4

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