Autoinflammatory syndromes
© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature..
The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increased inflammation parameters. Monogenic diseases include familial Mediterranean fever (FMF) and the newly described VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome. Heterogeneous diseases include adult-onset Still's disease and Schnitzler syndrome. Treatment is aimed at preventing the excessive inflammatory reaction in order to avoid long-term damage, such as amyloid A (AA) amyloidosis.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2023 |
|---|---|
| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:82 |
|---|---|
| Enthalten in: |
Zeitschrift fur Rheumatologie - 82(2023), 8 vom: 06. Okt., Seite 678-687 |
| Sprache: |
Deutsch |
|---|
| Weiterer Titel: |
Autoinflammatorische Syndrome |
|---|
| Beteiligte Personen: |
Bonnekoh, Hanna [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Autoinflammatory diseases |
|---|
| Anmerkungen: |
Date Revised 12.10.2023 published: Print Citation Status Publisher |
|---|
| doi: |
10.1007/s00393-023-01428-0 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM361697287 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM361697287 | ||
| 003 | DE-627 | ||
| 005 | 20231226085705.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2023 xx |||||o 00| ||ger c | ||
| 024 | 7 | |a 10.1007/s00393-023-01428-0 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1205.xml |
| 035 | |a (DE-627)NLM361697287 | ||
| 035 | |a (NLM)37672064 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a ger | ||
| 100 | 1 | |a Bonnekoh, Hanna |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Autoinflammatory syndromes |
| 246 | 3 | 3 | |a Autoinflammatorische Syndrome |
| 264 | 1 | |c 2023 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Revised 12.10.2023 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status Publisher | ||
| 520 | |a © 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature. | ||
| 520 | |a The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increased inflammation parameters. Monogenic diseases include familial Mediterranean fever (FMF) and the newly described VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome. Heterogeneous diseases include adult-onset Still's disease and Schnitzler syndrome. Treatment is aimed at preventing the excessive inflammatory reaction in order to avoid long-term damage, such as amyloid A (AA) amyloidosis | ||
| 650 | 4 | |a English Abstract | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Autoinflammatory diseases | |
| 650 | 4 | |a Familial Mediterranean fever | |
| 650 | 4 | |a Schnitzler syndrome | |
| 650 | 4 | |a Still’s disease, adult-onset | |
| 650 | 4 | |a VEXAS syndrome | |
| 700 | 1 | |a Krusche, Martin |e verfasserin |4 aut | |
| 700 | 1 | |a Feist, Eugen |e verfasserin |4 aut | |
| 700 | 1 | |a Wagner, Annette Doris |e verfasserin |4 aut | |
| 700 | 1 | |a Pankow, Anne |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Zeitschrift fur Rheumatologie |d 1974 |g 82(2023), 8 vom: 06. Okt., Seite 678-687 |w (DE-627)NLM000024287 |x 1435-1250 |7 nnns |
| 773 | 1 | 8 | |g volume:82 |g year:2023 |g number:8 |g day:06 |g month:10 |g pages:678-687 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1007/s00393-023-01428-0 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 82 |j 2023 |e 8 |b 06 |c 10 |h 678-687 | ||