This review evaluates PJP prophylaxis in rheumatic diseases, examining efficacy, adverse effects, and risk factors across conditions to support a risk-stratified, individualized approach to prophylaxis decisions.
Routine PJP prophylaxis is warranted in high-risk conditions (ANCA-associated vasculitis during induction, anti-MDA5+ dermatomyositis, VEXAS syndrome) especially with high-dose glucocorticoids, while low-risk conditions like Giant Cell Arteritis do not justify routine prophylaxis. PJP mortality in rheumatic disease exceeds that seen in HIV-infected patients.
Evidence-based guidelines specific to rheumatic disease are lacking; the review is narrative in design without systematic pooled data or meta-analysis, limiting quantitative conclusions.
Use a risk-stratified framework: prioritize PJP prophylaxis for patients with ANCA-associated vasculitis on induction therapy, anti-MDA5+ dermatomyositis, or VEXAS syndrome receiving high-dose glucocorticoids. Routine prophylaxis is not needed for all rheumatic disease patients — tailor decisions to individual risk.