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A new study published this week at the annual meeting of the American Academy of Rheumatology, ACR, shows the relationship between genotype, bone marrow failure, and the survival of patients with the rare autoinflammatory disease VEXAS syndrome, which is only in 2020.
Was found
.
VEXAS is an acronym for vacuole, E1 enzyme, x-linkage, auto-inflammation, and somatic syndrome.
It is a newly defined adult genetic disease that usually affects men
.
VEXAS is caused by mutations in the UBA1 gene responsible for the ubiquitin activating enzyme
This new study provides important clues for predicting the survival and mortality of patients with VEXAS syndrome
.
These factors have not been clearly defined before and can help guide doctors in the management of patients with this new disease
"Our interest in conducting this research is due to the high mortality of these patients with VEXAS syndrome
.
We want to determine the clinical features that predict survival, to understand the natural history of the disease, and to guide management decisions," said Marcela a.
The study included 73 patients who genetically confirmed VEXAS based on disease-related mutations in the UBA1 gene
.
Their median age at the time of onset was 66 years old, and the patients were between 40 and 85 years old
The researchers estimated the median survival rate of patients and compared the differences in survival rates of different genotypes
.
They also determined the clinical characteristics of patients (such as age of onset, thromboembolic disease, and pulmonary infiltration) as well as the correlation between transfusion dependence, genotype, and mortality
In total, they found that the patient mortality rate was 27%
.
The median survival age after onset is 10 years
The results of the study show that considering the high mortality rate of VEXAS syndrome and the current lack of effective medical treatment, the authors of the study believe that these patients should consider bone marrow transplantation, especially if they have other life-threatening risk factors
.
"Understanding specific variables related to survival can potentially help us decide which VEXAS syndrome patients may benefit from early and active intervention
.
It also helps us understand which parameters need to be followed when making these decisions
