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Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America
Sara E Sabbagh1,2, Jessica Neely3, Albert Chow4,5
1National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, MD, USA.
Insights
Pneumocystis jirovecii pneumonia (PJP) is a severe infection in juvenile idiopathic inflammatory myopathy (JIIM) patients, often linked to immunosuppressive therapy and specific clinical features. Prophylaxis may be considered for JIIM patients with anti-MDA5 autoantibodies, digital infarcts, skin ulcerations, or interstitial lung disease.
Area of Science:
- Pediatric Rheumatology
- Infectious Diseases
- Immunology
Background:
- Pneumocystis jirovecii pneumonia (PJP) poses significant risks in adult myositis patients.
- Limited research exists on PJP in juvenile idiopathic inflammatory myopathy (JIIM).
Purpose of the Study:
- To identify risk factors and clinical phenotypes associated with PJP in JIIM patients.
- To inform potential prophylactic strategies for high-risk JIIM individuals.
Main Methods:
- A REDCap questionnaire collected data from 13 PJP+ JIIM patients.
- Myositis features and medications were compared to 147 PJP- JIIM patients from NIH natural history studies.
Main Results:
- PJP+ patients had higher rates of Asian ancestry, anti-melanoma differentiation associated protein 5 (MDA5) autoantibodies, digital infarcts, skin ulcerations, and interstitial lung disease.
- PJP+ patients received more pulse steroids, rituximab, and overall immunosuppressive therapy prior to diagnosis.
- Seven PJP+ patients required intensive care, and four died due to PJP or its complications.
Conclusions:
- PJP is a severe, potentially fatal infection in JIIM.
- Risk factors include immunosuppressive therapy, anti-MDA5 autoantibodies, Asian ancestry, digital infarcts, cutaneous ulcerations, and interstitial lung disease.
- Consider PJP prophylaxis in JIIM patients exhibiting these risk factors.
Objectives:
Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, there are few studies examining PJP in juvenile myositis [juvenile idiopathic inflammatory myopathy (JIIM)]. The purpose of this study was to determine the risk factors and clinical phenotypes associated with PJP in JIIM.
Methods:
An research electronic data capture (REDCap) questionnaire regarding myositis features, disease course, medications and PJP infection characteristics was completed by treating physicians for 13 JIIM patients who developed PJP (PJP+) from the USA and Canada. Myositis features and medications were compared with 147 JIIM patients without PJP (PJP-) from similar geographic regions who enrolled in National Institutes of Health natural history studies.
Results:
PJP+ patients were more often of Asian ancestry than PJP- patients [odds ratio (OR) 8.7; 95% CI 1.3, 57.9]. Anti- melanoma differentiation associated protein 5 (MDA5) autoantibodies (OR 12.5; 95% CI 3.0, 52.4), digital infarcts (OR 43.8; 95% CI 4.2, 460.2), skin ulcerations (OR 12.0; 95% CI 3.5, 41.2) and interstitial lung disease (OR 10.6; 95% CI 2.1, 53.9) were more frequent in PJP+ patients. Before PJP diagnosis, patients more frequently received pulse steroids, rituximab and more immunosuppressive therapy compared with PJP- patients. Seven PJP+ patients were admitted to the intensive care unit and four patients died due to PJP or its complications.
Conclusions:
PJP is a severe infection in JIIM that can be associated with mortality. Having PJP was associated with more immunosuppressive therapy, anti-MDA5 autoantibodies, Asian race and certain clinical features, including digital infarcts, cutaneous ulcerations and interstitial lung disease. Prophylaxis for PJP should be considered in juvenile myositis patients with these features.
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