Adult-onset systemic autoinflammatory disorders: a clinical approach
Autoinflammatory disorders (AIDs) are a subgroup of immune-mediated syndromes that result from a primary dysfunction of the innate immune system. AIDs can be either monogenic or polygenic diseases. Unlike organspecific AIDs, systemic AIDs are characterized by fever and/or elevation of acute-phase re...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2020-01-01
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| Series: | Reumatismo |
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| Online Access: | https://www.reumatismo.org/index.php/reuma/article/view/1192 |
| _version_ | 1818475472913170432 |
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| author | T. Borges A. Barbosa S. Silva |
| author_facet | T. Borges A. Barbosa S. Silva |
| author_sort | T. Borges |
| collection | DOAJ |
| description | Autoinflammatory disorders (AIDs) are a subgroup of immune-mediated syndromes that result from a primary dysfunction of the innate immune system. AIDs can be either monogenic or polygenic diseases. Unlike organspecific AIDs, systemic AIDs are characterized by fever and/or elevation of acute-phase reactants. This review aims to describe the most common adult-onset systemic AIDs, focusing mostly on polygenic and mixed-pattern diseases which are expected to be more prevalent in adult patients than monogenic AIDs overall. The literature was searched in Medline database. Organ-specific or childhood-onset systemic AIDs were excluded. AIDs were divided in three distinct groups: mixed-pattern, polygenic and adult-onset monogenic AIDs. Most adult-onset AIDs are polygenic but late-onset disease is not rare among monogenic AIDs such as familial Mediterranean fever (FMF). The diagnosis of systemic AIDs in adults is often delayed due to several factors and sometimes it is only established when amyloidosis or other complications are present. Therefore, it probably makes sense to primarily exclude common AIDs in adult patients with fever of unknown origin (and probably different presentations such as polyserositis) since a high prevalence of adult-onset Still’s disease or FMF is usually expected. Colchicine, nonsteroidal anti-inflammatory drugs, steroids, immunosuppressive agents, interleukin-1 inhibitors and tumor necrosis factor antagonists constitute common therapeutic options for systemic AIDs. |
| first_indexed | 2024-12-10T09:13:01Z |
| format | Article |
| id | doaj.art-e2b94411db7d483f94c1e253a7ff6d5f |
| institution | Directory Open Access Journal |
| issn | 0048-7449 2240-2683 |
| language | English |
| last_indexed | 2024-12-10T09:13:01Z |
| publishDate | 2020-01-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Reumatismo |
| spelling | doaj.art-e2b94411db7d483f94c1e253a7ff6d5f2022-12-22T01:54:57ZengPAGEPress PublicationsReumatismo0048-74492240-26832020-01-0171410.4081/reumatismo.2019.1192Adult-onset systemic autoinflammatory disorders: a clinical approachT. Borges0A. Barbosa1S. Silva2Department of Internal Medicine, Trofa Saúde Hospital Privado de Gaia, Vila Nova de GaiaDepartment of Internal Medicine, Centro Hospitalar de São João, PortoDepartment of Internal Medicine, Trofa Saúde Hospital Privado de Gaia, Vila Nova de GaiaAutoinflammatory disorders (AIDs) are a subgroup of immune-mediated syndromes that result from a primary dysfunction of the innate immune system. AIDs can be either monogenic or polygenic diseases. Unlike organspecific AIDs, systemic AIDs are characterized by fever and/or elevation of acute-phase reactants. This review aims to describe the most common adult-onset systemic AIDs, focusing mostly on polygenic and mixed-pattern diseases which are expected to be more prevalent in adult patients than monogenic AIDs overall. The literature was searched in Medline database. Organ-specific or childhood-onset systemic AIDs were excluded. AIDs were divided in three distinct groups: mixed-pattern, polygenic and adult-onset monogenic AIDs. Most adult-onset AIDs are polygenic but late-onset disease is not rare among monogenic AIDs such as familial Mediterranean fever (FMF). The diagnosis of systemic AIDs in adults is often delayed due to several factors and sometimes it is only established when amyloidosis or other complications are present. Therefore, it probably makes sense to primarily exclude common AIDs in adult patients with fever of unknown origin (and probably different presentations such as polyserositis) since a high prevalence of adult-onset Still’s disease or FMF is usually expected. Colchicine, nonsteroidal anti-inflammatory drugs, steroids, immunosuppressive agents, interleukin-1 inhibitors and tumor necrosis factor antagonists constitute common therapeutic options for systemic AIDs.https://www.reumatismo.org/index.php/reuma/article/view/1192Autoinflammatory disordersinnate immunityadult. |
| spellingShingle | T. Borges A. Barbosa S. Silva Adult-onset systemic autoinflammatory disorders: a clinical approach Reumatismo Autoinflammatory disorders innate immunity adult. |
| title | Adult-onset systemic autoinflammatory disorders: a clinical approach |
| title_full | Adult-onset systemic autoinflammatory disorders: a clinical approach |
| title_fullStr | Adult-onset systemic autoinflammatory disorders: a clinical approach |
| title_full_unstemmed | Adult-onset systemic autoinflammatory disorders: a clinical approach |
| title_short | Adult-onset systemic autoinflammatory disorders: a clinical approach |
| title_sort | adult onset systemic autoinflammatory disorders a clinical approach |
| topic | Autoinflammatory disorders innate immunity adult. |
| url | https://www.reumatismo.org/index.php/reuma/article/view/1192 |
| work_keys_str_mv | AT tborges adultonsetsystemicautoinflammatorydisordersaclinicalapproach AT abarbosa adultonsetsystemicautoinflammatorydisordersaclinicalapproach AT ssilva adultonsetsystemicautoinflammatorydisordersaclinicalapproach |