Antiphospholipid syndrome pathogenesis in 2023: an update of new mechanisms or just a reconsideration of the old ones?
Rheumatology (Oxford)
; 63(SI): SI4-SI13, 2024 Feb 06.
Article
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| MEDLINE
| ID: mdl-38320591
ABSTRACT
Antibodies against phospholipid (aPL)-binding proteins, in particular, beta 2 glycoprotein I (ß2GPI), are diagnostic/classification and pathogenic antibodies in antiphospholipid syndrome (APS). ß2GPI-aPL recognize their target on endothelium and trigger a pro-thrombotic phenotype which is amplified by circulating monocytes, platelets and neutrophils. Complement activation is required as supported by the lack of aPL-mediated effects in animal models when the complement cascade is blocked. The final result is a localized clot. A strong generalized inflammatory response is associated with catastrophic APS, the clinical variant characterized by systemic thrombotic microangiopathy. A two-hit hypothesis was suggested to explain why persistent aPL are associated with acute events only when a second hit allows antibody/complement binding by modulating ß2GPI tissue presentation. ß2GPI/ß2GPI-aPL are also responsible for obstetric APS, being the molecule physiologically present in placental/decidual tissues. Additional mechanisms mediated by aPL with different characteristics have been reported, but their diagnostic/prognostic value is still a matter of research.
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Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Trombosis
/
Síndrome Antifosfolípido
Tipo de estudio:
Etiology_studies
/
Prognostic_studies
Idioma:
En
Revista:
Rheumatology (Oxford)
Asunto de la revista:
REUMATOLOGIA
Año:
2024
Tipo del documento:
Article