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Published on: April 25, 2016
Exploring glucocorticoid dose-response patterns in VEXAS syndrome: a pilot retrospective study
Nicolas Giachetti1, Jérome Hadjadj2,3, Noémie Gaudré4
1Médecine interne - Immunologie clinique, Centre National de Référence Maladies Auto-immunes Systémiques Rares du Nord, Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM-Angers), CHU d'Angers, Angers, France.
Glucocorticoid (GC) dose impacts VEXAS syndrome activity and flares. Higher GC doses correlate with lower disease activity, with flare risk increasing below 15 mg/day.
Area of Science:
- Rheumatology
- Immunology
- Pharmacology
Background:
- VEXAS syndrome requires high glucocorticoid (GC) doses.
- GC dose-response patterns in VEXAS syndrome are not well-defined.
- Frequent relapses occur during GC tapering.
Purpose of the Study:
- Assess feasibility of granular treatment-line reconstruction in VEXAS syndrome.
- Explore GC dose-response patterns related to disease activity.
- Investigate GC dose effects on flare risk.
Main Methods:
- Retrospective single-center pilot study.
- Reconstructed detailed treatment trajectories for 12 VEXAS patients.
- Analyzed GC dose, concomitant therapies, and clinical activity using multivariable models.
Main Results:
- Higher GC dose was independently associated with lower clinical activity (aOR 0.91).
- Flare risk increased below ~15 mg/day GC, with a plateau above 20 mg/day.
- Organ-specific GC sensitivity varied; lung involvement required higher doses.
Conclusions:
- Detailed treatment-line reconstruction is feasible in VEXAS syndrome.
- Identified GC dose-response trends and potential flare thresholds.
- Findings inform future studies on VEXAS syndrome GC tapering strategies.
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