Serious Lung Complication in sJIA Linked to Biologics

Published October 2019 | Arthritis & Rheumatology

For thousands of children living with systemic juvenile idiopathic arthritis (sJIA), a class of medications known as “biologics” have produced dramatic, life-changing improvements.

Now, however, experts at Cincinnati Children’s have documented 18 cases of a rare and potentially fatal lung complication that they call “systemic JIA-associated lung disease” or (sJIA-LD).

The children experiencing the lung problems tended to be less than 2 years old when their sJIA was diagnosed, their use of biologics often failed to push their disease into remission and eight of the 18 experienced adverse reactions to the medications. Importantly, 80% of the children with lung disease also had a past brush with macrophage activation syndrome (MAS), another dangerous complication of sJIA.

The 18 children identified here have been added to a national collection that exceeds 100 cases. The good news, early identification and aggressive supportive care appears to sharply reduce the risk of dying from this condition.

“The doctors taking care of these patients need to be more aware of this complication and have a very high index of suspicion for it,” says lead author Grant Schulert, MD, PhD. “Similarly, parents and families of these patients need to be talking to their doctors about this lung disease and making sure that their doctors are screening for it.”

Schulert and colleagues are continuing to study the complication and plan to identify a more-refined set of symptoms for doctors and families to watch for.