Abstract

BackgroundPneumocystis jiroveci pneumonia (PJP) is an opportunistic infection affecting immunocompromised individuals. However, evidence regarding the burden and effectiveness of prophylaxis among rheumatic patients remains limited. Delineating the epidemiology and efficacy of prophylaxis among rheumatic patients is urgently needed.MethodsWe performed a territory-wide cohort study of rheumatic patients in Hong Kong. All patients with a diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), immune-mediated myositis (IMM), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or spondyloarthritis (SpA) between 2015 and 2019 were included. Prevalence, frequency of prophylaxis and mortality of PJP were calculated. Number needed to treat (NNT) analysis was also performed.ResultsOut of 21,587 patients (54% RA, 25% SLE, 13% SpA, 5% IMM, 2% AAV and 1% SSc), 1141 (5.3%) patients were prescribed PJP prophylaxis. 48/21,587 (0.2%) developed PJP. No patients who developed PJP received prophylaxis prior to infection. The incidence of PJP was highest among SSc, AAV, and IMM patients. Among these diseases, the majority of PJP occurred while patients were on glucocorticoids at daily prednisolone-equivalent doses of 15 mg/day (P15) or above. PJP prophylaxis was effective with NNT for SSc, AAV and IIM being 36, 48 and 114 respectively. There were 19 PJP-related mortalities and the mortality rate was 39.6%.ConclusionPJP is an uncommon but important infection among rheumatic patients, PJP prophylaxis is effective and should be considered in patients with SSc, AAV and IMM, especially those receiving glucocorticoid doses above P15.

Highlights

  • Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection affecting immunocompromised individuals

  • *Correspondence: liphilip@hku.hk Division of Rheumatology and Clinical Immunology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China (HIV) but its incidence has declined over the past decades following the introduction of highly active antiretroviral therapy and routine PJP prophylaxis [1, 2]

  • Due to its high mortality, PJP prophylaxis is commonly prescribed in many immunocompromising conditions and various guidelines have been established in oncology, bone marrow and solid organ transplant (SOT) [5,6,7]

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Summary

Introduction

Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection affecting immunocompromised individuals. PJP was the most common cause of death among patients with human immunodeficiency virus (HIV) but its incidence has declined over the past decades following the introduction of highly active antiretroviral therapy and routine PJP prophylaxis [1, 2]. PJP has become an increasingly important cause of atypical pneumonia among non-HIV immunocompromised patients due to the expanding armamentarium in immunosuppressive and chemotherapeutic therapies [3]. PJP in non-HIV patients runs an aggressive course and is associated with even higher mortality compared with. Evidence regarding the burden and effectiveness of prophylaxis among rheumatic patients remains limited. Delineating the epidemiology and efficacy of prophylaxis among rheumatic patients is urgently needed

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