Date: Monday, November 9, 2020
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.
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 November 26, 2020.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-factors-associated-with-pneumocystis-jirovecii-pneumonia-in-juvenile-myositis-in-north-america/