This case provides key learning points, underscoring the heterogeneity and aggressiveness of CAPS, the importance of systematic exclusion of mimics when histopathology is not feasible, and the value of early "triple therapy" with escalation (e.g., rituximab) in refractory presentations. Large-vessel with cardiac extension (SVC→RA) in CAPS is uncommon and warrants heightened suspicion, even in young patients without conventional risk factors.
Skafi et al. (2026) studied this question.