APDS patients with immune-complex vasculitis and resolution with leniolisib.
Clin Immunol
; 262: 110176, 2024 May.
Article
en En
| MEDLINE
| ID: mdl-38462154
ABSTRACT
Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneous clinical manifestations of infections, immune dysregulation, autoimmunity; lymphoproliferation; and malignancy. Immune complex-mediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of immune complex-mediated vasculitis that activates complement and attracts neutrophils, macrophages and eosinophils to cause local tissue injury. Leniolisib is an inhibitor of PI3K p110δ and an FDA-approved treatment for APDS. IgA vasculitis resolved upon treatment with leniolisib. Patients with immune dysregulation including IgA vasculitis should be screened for APDS.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Asunto principal:
Poliarteritis Nudosa
/
Vasculitis por IgA
/
Piridinas
/
Pirimidinas
/
Arteritis de Células Gigantes
/
Granulomatosis con Poliangitis
/
Síndrome Mucocutáneo Linfonodular
Límite:
Humans
Idioma:
En
Revista:
Clin Immunol
/
Clin. immunol
/
Clinical immunology
Asunto de la revista:
ALERGIA E IMUNOLOGIA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Estados Unidos