The Great Pediatric Debate 2024: Cessation or Continuation of Biologics in sJIA Lung Disease?

Dr. Onel also noted anecdotally that she has never seen a patient with sJIA-related lung disease who had their sJIA truly well controlled before they developed lung symptoms. She added, “If someone is doing well on a biologic, I would say stopping treatment is a real problem.”

To counter, Dr. Cron underscored a recently published article which may help convince some people of the value of treatment cessation. The group retrospectively evaluated outcomes after physicians opted to stop or not stop an IL-1 or IL-6 inhibitor after an sJIA patient had an apparent DRESS response (the large majority of whom also had lung involvement).⁷

“It’s not a randomized trial, but it is data: If you develop lung disease in the setting of systemic JIA and you stop the offending agent—either the IL-1 or IL-6 inhibitor—you markedly improve survival,” said Dr. Cron. However, a single study of this nature cannot be definitive; although the two groups by chance ended up fairly balanced for risk factors, potential issues such as confounding by indication may still be at play.

In the end, the evidence may not yet definitively tip one way or the other, hence the need for a debate. After the talk, a poll revealed that audience members were evenly divided about cessation of IL-1/IL-6 inhibitors in this setting.

“We have to be cautious in both directions,” said Dr. Onel. “Like many questions in rheumatology, the answer is probably, ‘It depends.'”

Ruth Jessen Hickman, MD, a graduate of the Indiana University School of Medicine, is a medical and science writer in Bloomington, Ind.

References

1. Onel KB, Horton DB, Lovell DJ, et al. 2021 American College of Rheumatology guideline for the treatment of juvenile idiopathic arthritis: Therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2022;74(4):521–537.
2. Saper VE, Chen G, Deutsch GH, et al. Emergent high fatality lung disease in systemic juvenile arthritis. Ann Rheum Dis. 2019;78(12):1722–1731.
3. Schulert GS, Yasin S, Carey B, et al. Systemic juvenile idiopathic arthritis-associated lung disease: Characterization and risk factors. Arthritis Rheumatol. 2019;71(11):1943–1954. doi:10.1002/art.41073
4. Saper VE, Ombrello MJ, Tremoulet AH, et al. Severe delayed hypersensitivity reactions to IL-1 and IL-6 inhibitors link to common HLA-DRB1*15 alleles. Ann Rheum Dis. 2022;81(3):406–415.
5. Binstadt BA, Nigrovic PA. The conundrum of lung disease and drug hypersensitivity-like reactions in systemic juvenile idiopathic arthritis. Arthritis Rheumatol. 2022;74(7):1122–1131.
6. Seguin A, Galicier L, Boutboul D, Lemiale V, Azoulay E. Pulmonary involvement in patients with hemophagocytic lymphohistiocytosis. Chest. 2016;149(5):1294–1301.
7. Saper VE, Tian L, Verstegen RHJ, et al. Interleukin (IL)-1/IL-6-inhibitor-associated drug reaction with eosinophilia and systemic symptoms (DReSS) in systemic inflammatory illnesses. J Allergy Clin Immunol Pract. 2024;12(11):2996-3013.e7.