Pneumocystis prophylaxis in rheumatic disease.

Pneumocystis jirovecii pneumonia (PJP) is a rare and potentially fatal opportunistic infection in patients with rheumatic diseases with mortality rates exceeding those seen in HIV-infected patients. Although PJP prophylaxis is highly effective, evidence-based guidelines specific to rheumatic disease are lacking resulting in substantial variability in clinical practice. This review examines the existing PJP literature with a focus on efficacy of prophylaxis, adverse effects, and risk factors across rheumatic diseases to inform a pragmatic, risk-stratified approach to prophylaxis. Certain conditions including ANCA-associated vasculitis during induction therapy, anti-MD5+ dermatomyositis and VEXAS syndrome warrant routine prophylaxis in the setting of high-dose glucocorticoid use whereas others conditions, such as Giant Cell Arteritis, do not justify routine prophylaxis. This review provides a practical framework to assist the practicing clinician in making individualized decisions regarding PJP prophylaxis in rheumatic disease.