Failure to thrive, interstitial lung disease, and progressive digital necrosis with onset in infancy.
J Am Acad Dermatol
; 74(1): 186-9, 2016 Jan.
Article
em En
| MEDLINE
| ID: mdl-26584874
ABSTRACT
Key teaching points ⢠SAVI is a recently described interferonopathy resulting from constitutive action of STING and up-regulation of IFN-ß signaling. ⢠SAVI is characterized by facial erythema with telangiectasia, acral/cold-sensitive tissue ulceration and amputations, and interstitial lung disease. It has overlapping features with Aicardi-Goutières syndrome and familial chilblain lupus. ⢠Traditional immunosuppressive medications and biologic therapies appear to be of limited benefit, but JAK inhibitors may impact disease progression.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças Autoimunes
/
Interferon Tipo I
/
Dermatopatias Vasculares
/
Doenças Pulmonares Intersticiais
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article