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Int. J. Mol. Sci. 2016, 17(12), 2111; doi:10.3390/ijms17122111

Lung Involvement in Children with Hereditary Autoinflammatory Disorders

1
Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy
2
Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy
*
Author to whom correspondence should be addressed.
Academic Editor: Johannes Haybaeck
Received: 20 November 2016 / Revised: 10 December 2016 / Accepted: 12 December 2016 / Published: 15 December 2016
(This article belongs to the Special Issue Pneumonia: Pathogenesis, Diagnostics, Therapeutics, and Prevention)
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Abstract

Short-lived systemic inflammatory reactions arising from disrupted rules in the innate immune system are the operating platforms of hereditary autoinflammatory disorders (HAIDs). Multiple organs may be involved and aseptic inflammation leading to disease-specific phenotypes defines most HAIDs. Lungs are infrequently involved in children with HAIDs: the most common pulmonary manifestation is pleuritis in familial Mediterranean fever (FMF) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), respectively caused by mutations in the MEFV and TNFRSF1A genes, while interstitial lung disease can be observed in STING-associated vasculopathy with onset in infancy (SAVI), caused by mutations in the TMEM173 gene. The specific pleuropulmonary diseases may range from sub-clinical abnormalities during inflammatory flares of FMF and TRAPS to a severe life-threatening disorder in children with SAVI. View Full-Text
Keywords: autoinflammatory disorder; pleuritis; interstitial lung disease; child autoinflammatory disorder; pleuritis; interstitial lung disease; child
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Tarantino, G.; Esposito, S.; Andreozzi, L.; Bracci, B.; D’Errico, F.; Rigante, D. Lung Involvement in Children with Hereditary Autoinflammatory Disorders. Int. J. Mol. Sci. 2016, 17, 2111.

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