title: Lung Involvement in Children with Hereditary Autoinflammatory Disorders.
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 theMEFVandTNFRSF1Agenes, while interstitial lung disease can be observed in STING-associated vasculopathy with onset in infancy (SAVI), caused by mutations in theTMEM173gene. 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.
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy. giusyda16@hotmail.it.
authors.forename: Giusyda
authors.initials: G
authors.lastname: Tarantino
authors.affiliation: 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. susanna.esposito@unimi.it.
authors.forename: Susanna
authors.initials: S
authors.lastname: Esposito
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy. laurandreozzi@gmail.com.
authors.forename: Laura
authors.initials: L
authors.lastname: Andreozzi
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy. benedetta.bracci@gmail.com.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Bracci
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy. francscaderrico1403@gmail.com.
authors.forename: Francesca
authors.initials: F
authors.lastname: D'Errico
authors.affiliation: Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, 00168 Rome, Italy. donato.rigante@unicatt.it.
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
mesh_terms: D002648:Child; D010505:Familial Mediterranean Fever / Q000473:pathology; D056660:Hereditary Autoinflammatory Diseases / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000473:pathology*; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000378:metabolism
chemical_list: D047888:Receptors, Tumor Necrosis Factor, Type I
keywords: autoinflammatory disorder; child; interstitial lung disease; pleuritis