Pneumocystis jirovecii pneumonia prophylaxis in rare systemic autoimmune rheumatic diseases: a systematic review of randomised controlled trials within the European Reference Network on Connective Tissue and Musculoskeletal Diseases

Authors' conclusion

This systematic review reveals a profound lack of high-level evidence for PJP prophylaxis in non-RA SARDs, identifying only a single, underpowered RCT with a high risk of bias. Future research should transition from a universal management approach to a risk-stratified model that prioritises high-risk periods characterised by induction therapy or high-dose glucocorticoid use (≥20-30 mg/day) and underlying risk factors such as pre-existing interstitial lung disease. In the absence of definitive trial data, leveraging the multidisciplinary expertise of the European Reference Networks is essential to harmonise care and develop expert-led clinical guidelines that optimise patient safety across Europe.