Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

Sara E Sabbagh^{1

2}, Jessica Neely³, Albert Chow^{4

5}, Marietta DeGuzman^{6

7}, Jamie Lai^{6

7

8}, Svetlana Lvovich⁹, Tara McGrath^{10

11}, Maria Pereira^{6

7}, Iago Pinal-Fernandez^{1

12

13}, Jordan Roberts¹⁴, Kelly Rouster-Stevens^{15

16}, Heinrike Schmeling¹⁷, Anjali Sura^{18

19}, Gabriel Tarshish²⁰, Lori Tucker^{10

11}, Lisa G Rider²¹, Susan Kim³; CARRA JDM Workgroup and the Childhood Myositis Heterogeneity Study Group

Affiliations

¹ National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, MD, USA.
² Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA.
³ Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁴ Division of Pediatric Rheumatology, Department of Pediatrics, Seattle Children's Hospital, Seattle, WA, USA.
⁵ Division of Pediatric Rheumatology, Department of Pediatrics, Loma Linda University Children's Hospital, Loma Linda, CA, USA.
⁶ Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.
⁷ Division of Rheumatology, Texas Children's Hospital, Houston, TX, USA.
⁸ University of Colorado School of Medicine, Division of Pediatric Rheumatology, Children's Hospital Colorado, Aurora, CO, USA.
⁹ Section of Rheumatology, Department of Pediatrics, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, USA.
¹⁰ Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, BC, USA.
¹¹ BC Children's Hospital, Vancouver, BC, Canada.
¹² Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
¹³ Faculty of Health Sciences, Universitat Oberta de Catalunya, Barcelona, Spain.
¹⁴ Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
¹⁵ Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
¹⁶ Children's Healthcare of Atlanta, Atlanta, GA, USA.
¹⁷ Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
¹⁸ Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.
¹⁹ Department of Pediatrics, Upstate University Hospital, Syracuse, NY, USA.
²⁰ Division of Pediatric Rheumatology, Department of Pediatrics, Children's Hospital at Montefiore, Bronx, NY, USA.
²¹ Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health (NIH), Bethesda, MD, USA.

PMID: 32889531
PMCID: PMC7850515
DOI: 10.1093/rheumatology/keaa436

Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

Sara E Sabbagh et al. Rheumatology (Oxford). 2021.

. 2021 Feb 1;60(2):829-836.

doi: 10.1093/rheumatology/keaa436.

Authors

Affiliations

¹ National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, MD, USA.
² Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA.
³ Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁴ Division of Pediatric Rheumatology, Department of Pediatrics, Seattle Children's Hospital, Seattle, WA, USA.
⁵ Division of Pediatric Rheumatology, Department of Pediatrics, Loma Linda University Children's Hospital, Loma Linda, CA, USA.
⁶ Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.
⁷ Division of Rheumatology, Texas Children's Hospital, Houston, TX, USA.
⁸ University of Colorado School of Medicine, Division of Pediatric Rheumatology, Children's Hospital Colorado, Aurora, CO, USA.
⁹ Section of Rheumatology, Department of Pediatrics, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, USA.
¹⁰ Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, BC, USA.
¹¹ BC Children's Hospital, Vancouver, BC, Canada.
¹² Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
¹³ Faculty of Health Sciences, Universitat Oberta de Catalunya, Barcelona, Spain.
¹⁴ Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
¹⁵ Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
¹⁶ Children's Healthcare of Atlanta, Atlanta, GA, USA.
¹⁷ Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
¹⁸ Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.
¹⁹ Department of Pediatrics, Upstate University Hospital, Syracuse, NY, USA.
²⁰ Division of Pediatric Rheumatology, Department of Pediatrics, Children's Hospital at Montefiore, Bronx, NY, USA.
²¹ Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health (NIH), Bethesda, MD, USA.

PMID: 32889531
PMCID: PMC7850515
DOI: 10.1093/rheumatology/keaa436

Abstract

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.

Keywords: Pneumocystis jirovecii pneumonia; anti-MDA5 autoantibodies; interstitial lung disease; juvenile idiopathic inflammatory myopathies; myositis; opportunistic infections.

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Comment in

Comment on: Risk factors associated with pneumocystis jirovecii pneumonia in juvenile myositis in North America.
Yoshida T, Waki D. Yoshida T, et al. Rheumatology (Oxford). 2021 Sep 1;60(9):e337-e338. doi: 10.1093/rheumatology/keab411. Rheumatology (Oxford). 2021. PMID: 33961010 No abstract available.

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Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

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Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

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