Autoinflammatory & rare systemic diseases
Acute kidney injury, an underrecognized feature of VEXAS syndrome
Authors' conclusion
AKI is an underrecognized feature of VEXAS occurring in 25% of patients in this cohort. Age at diagnosis and CRP were associated with time to first AKI event during follow up. Plasma cell-rich interstitial nephritis was the most common histopathologic finding.
Study context
- Population
- Patients with genetically confirmed UBA1 mutation consistent with VEXAS syndrome (all white men, mean age 66.3 ± 8.6 years)all white men
- Participants
- 81 patients
- Outcome
- Incidence of AKI (time-to-first AKI event)
Methodological detailsmanual chart review, Cox regression
- Manual chart review
- Cox regression
Main finding
As reported in the abstract
Developed acute kidney injury (AKI) during follow-up, n (%)
20
25%
Cumulative incidence of AKI at 1 year
6.2% (0.80-11.3%) (95% CI)
Cumulative incidence of AKI at 3 years
16.7% (7.5-25.0%) (95% CI)
Cumulative incidence of AKI at 5 years
27.9% (14.9-38.9%) (95% CI)
Observational data: associations, not effects.
Further findings
AKI relapse · during follow-up
- AKI relapsed, % of cases
- 90%
- Median number of relapses
- 6
Risk factors for time-to-first AKI event (Cox regression)
- Age and baseline C-reactive protein (CRP) significantly associated with time-to-first AKI event
Kidney biopsy histopathology (6 patients)
- Plasma cell-rich interstitial nephritis, n
- 3
- Neutrophilic-rich interstitial inflammation, n
- 1
- Leukocytoclastic peritubular capillaritis, n
- 1
- Acute tubular injury, n
- 1
Death during follow-up · median follow-up 3.5 years
- Died, %
- 22%
Response of AKI to GC
- AKI responded well to GC but relapsed upon tapering
Original publication
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