Vexas

There are no PJP prophylaxis guidelines for rheumatic disease, so practice varies widely between clinicians. This review assembles what the evidence supports: prophylaxis clearly works, the harms are real enough to matter, and the number needed to treat swings from about 4 in VEXAS to over 900 in rheumatoid arthritis — which is why the underlying disease, not the steroid dose alone, should drive the decision.

Relapsing polychondritis is not one disease with variable severity. A French cohort splits it into three clusters whose mortality runs 4%, 13% and 58% — and the worst cluster is almost certainly VEXAS, which this review argues should be treated as a disease mimic rather than a subtype. Chondritis is absent at presentation in 40%, there is no confirmatory test, and no randomised trial has ever been conducted.