Abstract | PURPOSE:
STING-associated vasculopathy with onset in infancy (SAVI) is a new rare auto-inflammatory disease. The purpose of this study is to report new cases and summarize the manifestations and outcome of SAVI. METHODS: We made a retrospective analysis of three pediatric patients diagnosed with SAVI between March 2016 and July 2018 in Beijing Children's Hospital. RESULTS: CONCLUSIONS: New clinical aspect of diffuse alveolar hemorrhage is first reported to be associated with SAVI. Unsatisfactory therapeutic effects of tofacitinib are observed in this study and further evaluations are needed.
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Authors | Xiaolei Tang, Hui Xu, Chunju Zhou, Yun Peng, Hui Liu, Jinrong Liu, Huimin Li, Haiming Yang, Shunying Zhao |
Journal | Journal of clinical immunology
(J Clin Immunol)
Vol. 40
Issue 1
Pg. 114-122
(01 2020)
ISSN: 1573-2592 [Electronic] Netherlands |
PMID | 31705453
(Publication Type: Journal Article)
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Chemical References |
- Membrane Proteins
- Piperidines
- Pyrimidines
- STING1 protein, human
- tofacitinib
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Topics |
- Child, Preschool
- Female
- Hemorrhage
(drug therapy, genetics)
- Humans
- Infant
- Inflammation
(drug therapy, genetics)
- Lung
(drug effects)
- Lung Diseases, Interstitial
(drug therapy, genetics)
- Male
- Membrane Proteins
(genetics)
- Mutation
(genetics)
- Piperidines
(therapeutic use)
- Pyrimidines
(therapeutic use)
- Retrospective Studies
- Skin
(drug effects)
- Vascular Diseases
(drug therapy, genetics)
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