Review
Hereditary systemic autoinflammatory diseases
Enfermedades autoinflamatorias sistémicas hereditarias
Juan I. Aróstegui
Unidad de Enfermedades Autoinflamatorias, Servicio de Inmunología, Hospital Clínic, Villarroel, Barcelona, Spain
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"apellidos" => "Aróstegui" "email" => array:1 [ 0 => "jiaroste@clinic.ub.es" ] ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Unidad de Enfermedades Autoinflamatorias, Servicio de Inmunología, Hospital Clínic, Villarroel, Barcelona, Spain" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedades autoinflamatorias sistémicas hereditarias" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2010-01-07" "fechaAceptado" => "2010-01-10" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec112622" "palabras" => array:8 [ 0 => "Autoinflammatory diseases" 1 => "Hereditary periodic fever syndrome" 2 => "Innate immune response" 3 => "Inflammasome" 4 => "Nod-like receptor" 5 => "Pyrin" 6 => "Interleukin-1beta" 7 => "TNF" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec112623" "palabras" => array:8 [ 0 => "Enfermedades autoinflamatorias" 1 => "Síndromes hereditarios de fiebre periódica" 2 => "Respuesta inmune innata" 3 => "Inflamasoma" 4 => "Nod-like receptor" 5 => "Pirina" 6 => "Interleucina-1beta" 7 => "TNF" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Systemic autoinflammatory diseases encompass different rare clinical entities characterized by recurrent acute inflammatory episodes secondary to a dysregulated inflammatory process. Since their first clinical descriptions, the Mendelian hereditary nature of some of them became evident, with their genetic and molecular basis being recently elucidated. There are disease-causing mutations in genes encoding for different proteins involved in the innate immune response and inflammation. Herein, we will introduce the reader to an updated review of the main clinical, physiopathological and therapeutic features of the different hereditary systemic autoinflammatory diseases.</p>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Las enfermedades autoinflamatorias sistémicas engloban un conjunto de enfermedades poco frecuentes caracterizadas todas ellas por la presencia de episodios inflamatorios agudos y recurrentes, que son consecuencia de una disregulación del control del proceso inflamatorio. Desde sus respectivas descripciones clínicas, se había observado un claro patrón hereditario mendeliano para algunas de ellas. En fechas recientes se han identificado los defectos genéticos y moleculares subyacentes al identificarse mutaciones responsables de enfermedad en diferentes genes relacionados con la respuesta inmune innata y con la inflamación. A lo largo de la presente revisión se abordarán de una manera actualizada los principales aspectos clínicos, fisiopatológicos y terapéuticos de las diferentes enfermedades autoinflamatorias hereditarias.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:73 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 7 | 19 | 26 |
| 2024 October | 31 | 38 | 69 |
| 2024 September | 40 | 20 | 60 |
| 2024 August | 50 | 38 | 88 |
| 2024 July | 34 | 37 | 71 |
| 2024 June | 31 | 31 | 62 |
| 2024 May | 39 | 28 | 67 |
| 2024 April | 33 | 34 | 67 |
| 2024 March | 26 | 27 | 53 |
| 2024 February | 31 | 26 | 57 |
| 2024 January | 19 | 28 | 47 |
| 2023 December | 26 | 31 | 57 |
| 2023 November | 19 | 28 | 47 |
| 2023 October | 25 | 36 | 61 |
| 2023 September | 32 | 40 | 72 |
| 2023 August | 18 | 18 | 36 |
| 2023 July | 25 | 29 | 54 |
| 2023 June | 23 | 26 | 49 |
| 2023 May | 28 | 20 | 48 |
| 2023 April | 26 | 20 | 46 |
| 2023 March | 23 | 35 | 58 |
| 2023 February | 27 | 36 | 63 |
| 2023 January | 19 | 34 | 53 |
| 2022 December | 29 | 40 | 69 |
| 2022 November | 29 | 31 | 60 |
| 2022 October | 19 | 30 | 49 |
| 2022 September | 29 | 33 | 62 |
| 2022 August | 48 | 48 | 96 |
| 2022 July | 37 | 34 | 71 |
| 2022 June | 26 | 50 | 76 |
| 2022 May | 32 | 49 | 81 |
| 2022 April | 32 | 56 | 88 |
| 2022 March | 28 | 50 | 78 |
| 2022 February | 23 | 39 | 62 |
| 2022 January | 23 | 37 | 60 |
| 2021 December | 19 | 46 | 65 |
| 2021 November | 36 | 59 | 95 |
| 2021 October | 25 | 50 | 75 |
| 2021 September | 29 | 42 | 71 |
| 2021 August | 21 | 34 | 55 |
| 2021 July | 14 | 36 | 50 |
| 2021 June | 30 | 38 | 68 |
| 2021 May | 24 | 40 | 64 |
| 2021 April | 34 | 97 | 131 |
| 2021 March | 28 | 27 | 55 |
| 2021 February | 24 | 21 | 45 |
| 2021 January | 17 | 19 | 36 |
| 2020 December | 34 | 22 | 56 |
| 2020 November | 15 | 22 | 37 |
| 2020 October | 13 | 17 | 30 |
| 2020 September | 33 | 21 | 54 |
| 2020 August | 23 | 22 | 45 |
| 2020 July | 17 | 22 | 39 |
| 2020 June | 25 | 22 | 47 |
| 2020 May | 21 | 12 | 33 |
| 2020 April | 24 | 20 | 44 |
| 2020 March | 16 | 17 | 33 |
| 2020 January | 13 | 0 | 13 |
| 2019 September | 4 | 0 | 4 |
| 2019 June | 2 | 0 | 2 |
| 2019 March | 2 | 0 | 2 |
| 2019 January | 1 | 0 | 1 |
| 2018 May | 1 | 1 | 2 |
| 2018 April | 24 | 17 | 41 |
| 2018 March | 21 | 24 | 45 |
| 2018 February | 11 | 12 | 23 |
| 2018 January | 5 | 10 | 15 |
| 2017 December | 17 | 12 | 29 |
| 2017 November | 12 | 15 | 27 |
| 2017 October | 11 | 15 | 26 |
| 2017 September | 13 | 19 | 32 |
| 2017 August | 10 | 16 | 26 |
| 2017 July | 12 | 17 | 29 |
| 2017 June | 24 | 39 | 63 |
| 2017 May | 24 | 35 | 59 |
| 2017 April | 23 | 18 | 41 |
| 2017 March | 19 | 51 | 70 |
| 2017 February | 10 | 18 | 28 |
| 2017 January | 15 | 12 | 27 |
| 2016 December | 32 | 35 | 67 |
| 2016 November | 27 | 24 | 51 |
| 2016 October | 48 | 21 | 69 |
| 2016 September | 33 | 14 | 47 |
| 2016 August | 20 | 10 | 30 |
| 2016 July | 18 | 7 | 25 |
| 2016 June | 0 | 11 | 11 |
| 2016 May | 1 | 7 | 8 |
| 2016 April | 0 | 12 | 12 |
| 2016 February | 0 | 18 | 18 |
| 2015 December | 9 | 0 | 9 |
| 2015 November | 1 | 12 | 13 |
| 2015 September | 9 | 0 | 9 |
| 2015 August | 0 | 10 | 10 |
| 2015 July | 10 | 5 | 15 |
| 2015 June | 33 | 12 | 45 |
| 2015 May | 42 | 34 | 76 |
| 2015 April | 27 | 25 | 52 |
| 2015 March | 18 | 12 | 30 |
| 2015 February | 26 | 26 | 52 |
| 2015 January | 24 | 30 | 54 |
| 2014 December | 30 | 23 | 53 |
| 2014 November | 23 | 19 | 42 |
| 2014 October | 26 | 25 | 51 |
| 2014 September | 30 | 10 | 40 |
| 2014 August | 23 | 24 | 47 |
| 2014 July | 20 | 27 | 47 |
| 2014 June | 44 | 19 | 63 |
| 2014 May | 43 | 20 | 63 |
| 2014 April | 35 | 12 | 47 |
| 2014 March | 48 | 30 | 78 |
| 2014 February | 33 | 22 | 55 |
| 2014 January | 33 | 22 | 55 |
| 2013 December | 32 | 8 | 40 |
| 2013 November | 29 | 22 | 51 |
| 2013 October | 46 | 23 | 69 |
| 2013 September | 38 | 19 | 57 |
| 2013 August | 36 | 28 | 64 |
| 2013 July | 32 | 20 | 52 |
| 2013 June | 29 | 22 | 51 |
| 2013 May | 37 | 19 | 56 |
| 2013 April | 29 | 28 | 57 |
| 2013 March | 21 | 25 | 46 |
| 2013 February | 35 | 20 | 55 |
| 2013 January | 22 | 21 | 43 |
| 2012 December | 25 | 18 | 43 |
| 2012 November | 18 | 16 | 34 |
| 2012 October | 11 | 13 | 24 |
| 2012 September | 7 | 7 | 14 |
| 2012 July | 3 | 0 | 3 |
| 2012 June | 3 | 0 | 3 |
| 2012 April | 4 | 0 | 4 |
| 2012 March | 2 | 0 | 2 |
| 2012 February | 2 | 0 | 2 |
| 2012 January | 4 | 0 | 4 |
| 2011 December | 10 | 0 | 10 |
| 2011 November | 2 | 0 | 2 |
| 2011 October | 4 | 0 | 4 |
| 2011 July | 1 | 0 | 1 |
| 2011 June | 2 | 0 | 2 |
| 2011 May | 2 | 0 | 2 |
| 2011 January | 12 | 0 | 12 |
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