What we know and what we don't know about catastrophic antiphospholipid syndrome
© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissionsoup.com..
Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:63 |
|---|---|
| Enthalten in: |
Rheumatology (Oxford, England) - 63(2024), SI vom: 06. Feb., Seite SI46-SI53 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Rodriguez-Pintó, Ignasi [VerfasserIn] |
|---|
| Links: |
|---|
| Anmerkungen: |
Date Completed 08.02.2024 Date Revised 08.02.2024 published: Print Citation Status MEDLINE |
|---|
| doi: |
10.1093/rheumatology/kead556 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM368099709 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM368099709 | ||
| 003 | DE-627 | ||
| 005 | 20240208232114.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 240207s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1093/rheumatology/kead556 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1284.xml |
| 035 | |a (DE-627)NLM368099709 | ||
| 035 | |a (NLM)38320593 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Rodriguez-Pintó, Ignasi |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a What we know and what we don't know about catastrophic antiphospholipid syndrome |
| 264 | 1 | |c 2024 | |
| 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 Completed 08.02.2024 | ||
| 500 | |a Date Revised 08.02.2024 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissionsoup.com. | ||
| 520 | |a Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition | ||
| 650 | 4 | |a Review | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a anticoagulation | |
| 650 | 4 | |a catastrophic antiphospholipid syndrome | |
| 650 | 4 | |a cytokine storm | |
| 650 | 4 | |a eculizumab | |
| 650 | 4 | |a glucocorticoids | |
| 650 | 4 | |a intravenous immunoglobulins | |
| 650 | 4 | |a plasma exchange | |
| 650 | 4 | |a rituximab | |
| 650 | 4 | |a therapeutics | |
| 650 | 4 | |a thrombotic microangiopathy | |
| 650 | 7 | |a Immunoglobulins, Intravenous |2 NLM | |
| 700 | 1 | |a Espinosa, Gerard |e verfasserin |4 aut | |
| 700 | 1 | |a Cervera, Ricard |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Rheumatology (Oxford, England) |d 1999 |g 63(2024), SI vom: 06. Feb., Seite SI46-SI53 |w (DE-627)NLM102581908 |x 1462-0332 |7 nnns |
| 773 | 1 | 8 | |g volume:63 |g year:2024 |g number:SI |g day:06 |g month:02 |g pages:SI46-SI53 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1093/rheumatology/kead556 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 63 |j 2024 |e SI |b 06 |c 02 |h SI46-SI53 | ||