Faculty Collaboration Database

Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America. Rheumatology (Oxford) 2021 Feb 01;60(2):829-836

Date

09/06/2020

Pubmed ID

32889531

Pubmed Central ID

PMC7850515

DOI

10.1093/rheumatology/keaa436

Scopus ID

2-s2.0-85102212098 (requires institutional sign-in at Scopus site)   14 Citations

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.

Author List

Sabbagh SE, Neely J, Chow A, DeGuzman M, Lai J, Lvovich S, McGrath T, Pereira M, Pinal-Fernandez I, Roberts J, Rouster-Stevens K, Schmeling H, Sura A, Tarshish G, Tucker L, Rider LG, Kim S, CARRA JDM Workgroup and the Childhood Myositis Heterogeneity Study Group

Author

Sara Sabbagh DO Assistant Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Autoantibodies
Child
Dermatomyositis
Female
Humans
Immunosuppressive Agents
Interferon-Induced Helicase, IFIH1
Lung Diseases, Interstitial
Male
North America
Opportunistic Infections
Pneumonia, Pneumocystis
Risk Assessment
Risk Factors
Skin Ulcer