Risk Factors Associated with Pneumocystis Jirovecii Pneumonia in Juvenile Myositis in North America

Sara Sabbagh¹, Jessica Neely², Albert Chow³, Marietta DeGuzman⁴, Jamie Lai⁵, Svetlana Lvovich⁶, Tara McGrath⁷, Maria Pereira⁸, Iago Pinal-Fernandez⁹, Jordan Roberts¹⁰, Kelly Rouster-Stevens¹¹, Heinrike Schmeling¹², Anjali Sura¹³, Gabriel Tarshish¹⁴, Lori Tucker¹⁵, Lisa G. Rider¹⁶ and Susan Kim¹⁷, ¹Medical College of Wisconsin, Fox Point, WI, ²UCSF, San Francisco, CA, ³Loma Linda University, San Bernardino, CA, ⁴Baylor College of Medicine, Houston, ⁵University of Colorado, The Woodlands, TX, ⁶St Christopher's Hospital for Children, Philadelphia, PA, ⁷University of British Columbia, Vancouver, BC, Canada, ⁸Baylor College of Medicine, Houston, TX, ⁹National Institutes of Health, Bethesda, MD, ¹⁰Boston Children's Hospital, Boston, MA, ¹¹Emory University/Children's Healthcare of Atlanta, Atlanta, GA, ¹²University of Calgary, Calgary, AB, Canada, ¹³Upstate University Hospital, Syracuse, NY, ¹⁴Children's Hospital at Montefiore, New York, NY, ¹⁵BC Children's Hospital, Vancouver, BC, Canada, ¹⁶Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Garrett Park, MD, ¹⁷UCSF Benioff Children's Hospital, San Francisco, CA

Meeting: ACR Convergence 2020

Keywords: Autoantibody(ies), Infection, Myositis, Pediatric rheumatology, pulmonary

Session Information

Date: Monday, November 9, 2020

Title: Pediatric Rheumatology – Clinical Poster III: SLE, Vasculitis, & JDM

Session Type: Poster Session D

Session Time: 9:00AM-11:00AM

Background/Purpose: Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, few studies have examined PJP in juvenile myositis (JIIM). The purpose of this study was to determine risk factors and clinical phenotypes associated with PJP in JIIM.

Methods: An electronic 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 United States and Canada. Myositis features and medications were compared to 147 JIIM patients without PJP (PJP[-]) from similar geographic regions who enrolled in National Institutes of Health natural history studies.

Results: The median age at PJP diagnosis was 6.1 years (IQR 2.6-10.5). PJP occurred shortly after myositis diagnosis in the majority of patients, with a median time from JIIM diagnosis to PJP diagnosis of 2.3 months (IQR 1.7-7). At PJP diagnosis, overall JIIM disease severity was rated as moderate or severe in 10/13 patients. PJP[+] patients were more often of Asian ancestry than PJP[-] patients (OR 8.7; 95% CI 1.3-57.9). Anti-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 (ILD) (OR 10.6; 95% CI 2.1-53.9) were more frequent in PJP[+] than PJP[-] patients. Before PJP diagnosis, patients more frequently received pulse steroids (OR 3.8; 95% CI 1.2-12.4), rituximab (OR 52; 95% CI 5.2-515.4), and a greater number of immunosuppressive therapies, including corticosteroids, immunosuppressive drugs, and/or rituximab (OR 2.4; 95% CI 1.3-4.5) compared to PJP[-] patients; daily corticosteroid dose, however, did not differ between PJP[+] and PJP[-] patients. Seven PJP[+] patients were admitted to the intensive care unit, and four patients died due to PJP or its complications.

Conclusion: We identified that PJP more often affects JIIM patients early in their disease course, when patients have more severe manifestations requiring more intensive therapy. Importantly, we also identified anti-MDA5 autoantibodies, digital infarcts, skin ulcerations, and ILD as risk factors for developing PJP infection in JIIM. Thus, prophylaxis should be strongly considered in JIIM patients, especially those early in the disease course with these clinical features, and those who have received IVMP and multiple immunosuppressive therapies, particularly rituximab.

Disclosure: S. Sabbagh, None; J. Neely, None; A. Chow, None; M. DeGuzman, None; J. Lai, None; S. Lvovich, None; T. McGrath, None; M. Pereira, None; I. Pinal-Fernandez, None; J. Roberts, None; K. Rouster-Stevens, None; H. Schmeling, None; A. Sura, None; G. Tarshish, None; L. Tucker, None; L. Rider, NIEHS, NIH, 2, Cure JM Foundation, 2, Bristol Myers Squibb, 2, Hope Pharmaceuticals, 2, Eli Lilly and Company, 9, MedImmune/AstraZeneca, 9; S. Kim, None.

To cite this abstract in AMA style:

Sabbagh S, 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 L, Kim S. Risk Factors Associated with Pneumocystis Jirovecii Pneumonia in Juvenile Myositis in North America [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/risk-factors-associated-with-pneumocystis-jirovecii-pneumonia-in-juvenile-myositis-in-north-america/. Accessed .

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