| Title |
Familial Chilblain Lupus - What Can We Learn from Type I Interferonopathies?
|
|---|---|
| Published in |
Current Rheumatology Reports, August 2017
|
| DOI | 10.1007/s11926-017-0689-x |
| Pubmed ID | |
| Authors |
Christoph Fiehn |
| Abstract |
Familial chilblain lupus belongs to the group of type I interferonopathies and is characterized by typical skin manifestations and acral ischaemia. This review aims to give an overview of clinical signs and the pathophysiological mechanisms. There are several mutations that can lead to this autosomal dominant disease. Most frequent is a mutation of the gene for TREX-1. However, as well cases of families with mutations in the SAMHD1 gene and, recently, with one for the gene that codes for the protein stimulator of interferon genes have been described. These genes are involved in the process of the detection of intracellular DNA, and their mutation results in an increased production of type I interferons and their gene products, resulting in auto-inflammation and auto-immunity. JAK inhibitors have been successfully used to treat this disorder. Familial chilblain is a rare disorder with very distinct clinical signs. Its pathophysiological mechanism gives insight into the process of interferon-induced inflammation in auto-immune diseases. |
X Demographics
The data shown below were collected from the profile of 1 X user who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Spain | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 26 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 26 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 5 | 19% |
| Other | 4 | 15% |
| Student > Doctoral Student | 4 | 15% |
| Researcher | 4 | 15% |
| Professor | 2 | 8% |
| Other | 6 | 23% |
| Unknown | 1 | 4% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 8 | 31% |
| Biochemistry, Genetics and Molecular Biology | 4 | 15% |
| Pharmacology, Toxicology and Pharmaceutical Science | 2 | 8% |
| Unspecified | 1 | 4% |
| Immunology and Microbiology | 1 | 4% |
| Other | 3 | 12% |
| Unknown | 7 | 27% |
Attention Score in Context
This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 28 August 2017.
All research outputs
#18,569,430
of 22,999,744 outputs
Outputs from Current Rheumatology Reports
#563
of 718 outputs
Outputs of similar age
#242,598
of 316,465 outputs
Outputs of similar age from Current Rheumatology Reports
#12
of 21 outputs
Altmetric has tracked 22,999,744 research outputs across all sources so far. This one is in the 11th percentile – i.e., 11% of other outputs scored the same or lower than it.
So far Altmetric has tracked 718 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.4. This one is in the 10th percentile – i.e., 10% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 316,465 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 12th percentile – i.e., 12% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 21 others from the same source and published within six weeks on either side of this one. This one is in the 23rd percentile – i.e., 23% of its contemporaries scored the same or lower than it.