Researchers Alarmed By Lung Disease In Juvenile Arthritis Patients

Nov 11, 2019

A serious lung complication for a small group of juvenile arthritis patients is causing concern for both Cincinnati Children's Hospital researchers and the families of patients.

It was six years ago when one of Rashmi Sinha's one-year-old twins first showed signs of the rarest form of juvenile idiopathic arthritis, also called systemic JIA. It's a debilitating disease that affects thousands of children. The standard of care is a class of medications called biologics.

That's what her son Vikram was taking. Then, one year after his initial diagnoses, her son experienced chronic lung problems, a recent and disturbing trend with systemic JIA patients. At the time, the Sinhas were living in California and Vikram was in and out of the hospital.

"You can't have a peaceful moment once something like this has happened, and then I started meeting other parents who are going through the same nightmare scenario," says Sinha.

Eventually she started the national Systemic JIA Foundation to support parents and researchers. She moved to Cincinnati where Children's Hospital Medical Center doctors were already aware of this lung complication and treating it. 

Rashmi Sinha, a parent of a systemic JIA patient, talks to rheumatology expert Grant Schulert at Cincinnati Children's.
Credit Ann Thompson / WVXU

The cause is unclear. Children's rheumatology expert Grant Schulert says it's some combination of genetic risk factors and a trigger in the environment - it maybe an infection which sets this off. Biologic medications may also play a role.

Unfortunately, Schulert says there aren't any great alternatives to the genetically engineered medications. He says generally the patients at risk of getting the lung complication were diagnosed at age two or younger and have high levels of inflammation.

"We recommend that they get chest x-rays probably on an annual basis; that they have ultrasounds of their heart and other tests to look at their oxygen levels," says Schulert, 

Schulert is working with his colleagues here at Cincinnati Children's and across the country to discover the cause. There are hints of what's going on and it has to do with a particular kind of inflammation. Over the weekend he presented his findings at a conference in Atlanta.