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Updated: Jun 7, 2026

Microbiological Rapid On-Site Evaluation for Pulmonary Infectious Diseases
Published on: March 1, 2024
Pneumocystis prophylaxis in rheumatic disease
Rachel M Wolfe1, Kevin L Winthrop2
1Wake Forest School of Medicine, Section on Rheumatology, Medical Center Blvd, Winston Salem, NC, 27104, USA.
Abstract:
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.
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