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

ADOBE STOCK | Feodora

Washington D.C.—At a Sunday, Nov. 17, Pediatrics Great Debate session of ACR Convergence, speakers argued whether patients with systemic juvenile idiopathic arthritis (sJIA) should continue their interleukin (IL) 1/IL-6 biologics if lung disease is suspected.

Randy Q. Cron, MD, PhD, the director of the Division of Pediatric Rheumatology at the University of Alabama at Birmingham, made the case for discontinuing IL-1 or IL-6 inhibitors if lung disease is present.

In contrast, Karen B. Onel, MD, professor of clinical pediatrics at Weill Cornell Medicine and the chief of Pediatric Rheumatology at the Hospital for Special Surgery, New York City, argued for continuing standard sJIA biologic therapies, at least in some patients.

Dr. Karen Onel

Evolution of Treatment Options in sJIA

Both speakers emphasized the devastating nature and intense morbidity of untreated or inadequately treated sJIA. Dr. Onel pointed out that drugs used to treat the condition in the 20th and early 21st century were largely ineffective, and many were quite toxic.

But the advent of IL-1 and IL-6 inhibitors dramatically improved the situation for patients with sJIA. “When anakinra came out, it was nothing short of miraculous,” shared Dr. Cron. The biologics targeting IL-1 (anakinra, canakinumab and rilonacept) and IL-6 (tocilizumab) were well tolerated and highly effective, and clinicians quickly adopted them.

Current ACR guidelines recommend IL-1 or IL-6 inhibitors as first-line agents for sJIA patients, in both those who have or don’t have concurrent macrophage activation syndrome (MAS). Other biologics such as TNF inhibitors or conventional synthetic disease-modifying anti-rheumatic drugs can be utilized in patients with residual arthritis on IL-1 or IL-6 inhibitors. Current ACR guidelines do not directly speak to the management of sJIA patients on IL-1 or IL-6 inhibitors who develop lung disease.¹

Lung Disease in sJIA & Biologic Cessation

The lack of complete understanding about the etiology of sJIA-related lung disease is part of what makes management decisions challenging.

Before the advent of IL-1 and IL-6 inhibitors, lung disease related to sJIA was generally described as such findings as pleuritis or pleural effusion, although the literature reflected a few isolated case reports of other types of lung disease. However, in the years after the introduction of IL-1 and IL-6 inhibitors, studies have indicated increasing numbers of patients with sJIA-related lung disease, with lung diagnoses rarely reported previously, including pulmonary arterial hypertension, interstitial lung disease and pulmonary alveolar proteinosis. Although still considered rare, when it occurs the disease is often fatal.^2,3