Abstract

Pneumocystis jirovecii pneumonia (PJP) is an uncommon opportunistic infection in patients with rheumatic diseases with high mortality. Unlike other non-HIV conditions, international guideline for PJP prophylaxis in rheumatic diseases is currently lacking. Recent evidence regarding the risk of PJP and effectiveness of prophylaxis has been accumulating. This Review provides an update on the information about risk factors associated with PJP in patients with rheumatic diseases based on rheumatic diagnoses, use of immunosuppressive agents and other disease-related factors. The second part of the article summarizes evidence regarding the effectiveness of PJP prophylaxis by considering both disease-related and therapy-related factors. Finally, the Review outlined the currently available disease-specific recommendations and local guidelines, and appreciate the factors that influence physicians’ decision.

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