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.
Abstract

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