Drug Reaction With Eosinophilia and Systemic Symptoms Linked to High Fatality Lung Disease in JIA

Illustration comparing a normal knee joint (left) and arthritic knee joint (right). The disease commonly occurs between ages 7 to 12 and affects 1 in 1000 children each year. It is known as systemic juvenile idiopathic arthritis.
Drug reaction with eosinophilia and systemic symptoms (DReSS) with exposure to interleukin-1 or interleukin-6 inhibition may be linked to high fatality lung disease in systemic juvenile idiopathic arthritis.

After careful consideration, the American Academy of Allergy, Asthma & Immunology canceled its annual meeting that was to take place in Philadelphia, Pennsylvania from March 13 to 16, because of concerns regarding the coronavirus disease 2019 (COVID-19) outbreak. Although the live events will not proceed as planned, our readers can still find coverage of research that was scheduled to be presented at the meeting.

Drug reaction with eosinophilia and systemic symptoms (DReSS) with exposure to interleukin (IL)-1 or IL-6 inhibition may be linked to high fatality lung disease and poor outcomes in systemic juvenile idiopathic arthritis (sJIA), according to research intended to be presented at the American Academy of Allergy Asthma and Immunology (AAAAI) annual meeting.

Using REDCap databases, researchers collected retrospective observational data for 63 patients with sJIA with lung disease who were diagnosed by 55 physicians from 39 institutions. Researchers evaluated DReSS in patients by using the validated RegiSCAR scoring system.

Of the 63 patients with sJIA and lung disease, 48 had unusual high fatality lung disease associated with IL-1 or IL-6 inhibition. A total of 15 patients were scored with “definite” DReSS (score, >5/9) and 8 with “probable” DReSS (score, 4-5/9). At the time of eosinophilia diagnosis, all 23 patients had been exposed to ≥1 medications, including anakinra, tocilizumab, canakinumab, and rilonacept. Despite concurrent steroid treatment (0-2 mg/kg/d), median peak peripheral eosinophil count was 1725 (range, 754-13,156; 6%-45% of white blood cells) in patients with definite DReSS; facial rash with angioedema was reported in 12 patients.

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Among patients in the definite DReSS group, 8 patients stopped the suspected medication, of whom 6 survived (median survival since lung disease diagnosis, 4.7 years) and 2 died after restarting the medication. The remaining 7 patients who continued the medication died.

Overall, the researchers concluded that, “DReSS with lung involvement is a high fatality illness. Unrecognized DReSS with exposure to anti-IL-1/IL-6 may account for lung disease and poor outcome in some sJIA cases.”


Saper V, Mellins E, Kwong B. Drug reaction and high fatality lung disease in systemic onset juvenile idiopathic arthritis (sJIA). J Allergy Clin Immunol. 2020;145(Suppl 2):AB95.

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This article originally appeared on Pulmonology Advisor