VEXAS syndrome (notice n° 174711)
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| 000 -LEADER | |
|---|---|
| fixed length control field | 01434cam a2200193 4500500 |
| 005 - DATE AND TIME OF LATEST TRANSACTION | |
| control field | 20250112040002.0 |
| 041 ## - LANGUAGE CODE | |
| Language code of text/sound track or separate title | fre |
| 042 ## - AUTHENTICATION CODE | |
| Authentication code | dc |
| 100 10 - MAIN ENTRY--PERSONAL NAME | |
| Personal name | Laurent, Charlotte |
| Relator term | author |
| 245 00 - TITLE STATEMENT | |
| Title | VEXAS syndrome |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. | |
| Date of publication, distribution, etc. | 2022.<br/> |
| 500 ## - GENERAL NOTE | |
| General note | 33 |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | VEXAS syndrome (“vacuoles, E1 Enzyme, X-linked, autoinflammatory, somatic syndrome”) is a recently described autoinflammatory syndrome related to somatic mutations of UBA1. The main clinical features of VEXAS syndrome are heterogeneous, including relapsing fever, relapsing chondritis, and skin lesions such as neutrophilic dermatosis, lung and ocular involvement, venous thrombosis, lymph nodes, and arthralgia. VEXAS syndrome is associated with hematological disease in 50% of cases, mainly myelodysplastic syndrome and monoclonal gammopathy. Treatment depends on whether or not the disease is associated with a hematological disease. When VEXAS syndrome is associated with a myelodysplastic syndrome, hematological treatments, in particular azacitidine, seem to be among the most effective. In the absence of hematological disease, JAK inhibitors appear to be the most effective. |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Jachiet, Vincent |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Fain, Olivier |
| Relator term | author |
| 700 10 - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Mekinian, Arsène |
| Relator term | author |
| 786 0# - DATA SOURCE ENTRY | |
| Note | Hématologie | 28 | 6 | 2022-11-01 | p. 291-299 | 1264-7527 |
| 856 41 - ELECTRONIC LOCATION AND ACCESS | |
| Uniform Resource Identifier | <a href="https://shs.cairn.info/journal-hematologie-2022-6-page-291?lang=en">https://shs.cairn.info/journal-hematologie-2022-6-page-291?lang=en</a> |
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