Interferon alpha treatment of patients with impaired interferon gamma signaling.
J Clin Immunol
; 33(5): 991-1001, 2013 Jul.
Article
in En
| MEDLINE
| ID: mdl-23512243
ABSTRACT
Patients with deficiency in the interferon gamma receptor (IFN-γR) are unable to respond properly to IFN-γ and develop severe infections with nontuberculous mycobacteria (NTM). IFN-γ and IFN-α are known to signal through STAT1 and activate many downstream effector genes in common. Therefore, we added IFN-α for treatment of patients with disseminated mycobacterial disease in an effort to complement their IFN-γ signaling defect. We treated four patients with IFN-γR deficiency with adjunctive IFN-α therapy in addition to best available antimicrobial therapy, with or without IFN-γ, depending on the defect. During IFN-α treatment, ex vivo induction of IFN target genes was detected. In addition, IFN-α driven gene expression in patients' cells and mycobacteria induced cytokine response were observed in vitro. Clinical responses varied in these patients. IFN-α therapy was associated with either improvement or stabilization of disease. In no case was disease exacerbated. In patients with profoundly impaired IFN-γ signaling who have refractory infections, IFN-α may have adjunctive anti-mycobacterial effects.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Interferon-gamma
/
Interferon-alpha
/
Receptors, Interferon
Limits:
Adult
/
Child, preschool
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
J Clin Immunol
Year:
2013
Document type:
Article
Affiliation country:
United States