Immunological and therapeutic insights in autoimmune autonomic ganglionopathy: What is the position of apheresis in immunotherapy?
Transfus Apher Sci
; 63(4): 103967, 2024 Jul 02.
Article
en En
| MEDLINE
| ID: mdl-38959810
ABSTRACT
Autoimmune autonomic ganglionopathy (AAG) is characterized by various autonomic and extra-autonomic symptoms and is caused by autoantibodies against nicotinic acetylcholine receptors present in the autonomic ganglia (ganglionic acetylcholine receptor, gAChR), requiring immediate and aggressive intervention to prevent the exacerbation of symptoms. However, there is currently no internationally accepted standard of care for the immunotherapy of AAG, including apheresis. Although the rationale for the use of plasma exchange (PLEX) in AAG is strong, whereby pathogenic gAChR antibodies are removed, its overall impact on patient outcomes is not well-established. Based on previous case reports and small case series studies, we provide a comprehensive overview of the challenges and uncertainties surrounding the use of PLEX for the management of AAG and provide current practice recommendations to guide treatment decisions.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Idioma:
En
Revista:
Transfus Apher Sci
Asunto de la revista:
HEMATOLOGIA
Año:
2024
Tipo del documento:
Article