Potential Link Between a Treatment for Systemic Juvenile Idiopathic Arthritis and Lung Disease

The number of systemic juvenile idiopathic arthritis patients who develop lung disease (sJIA-LD) is rising in both the U.S., Europe, the Middle East, and Canada. This trend has been notable since 2010. Before that time, it was not unheard of but it was extremely rare. Elizabeth Mellins from Stanford and Grant Schulert from Cincinnati Children’s Hospital Medical Center set to find out why this condition is on the rise.

The uncanny thing is that the increasing presence of lung disease seems to be accompanying the widespread and long-term use of interleukin-1/IL-6 inhibition therapy, a treatment which has finally been allowing children with sJIA-LD to thrive and live a happy, normal life.

Dr. Mellins had a total of 121 patients in her investigation and Dr. Schulert had a total of 27, all of whom had the lung disease. In both of these studies, some of the patients improved, continued the standard therapy, and had no further lung issues. Other patients sadly succumbed to the disease.

These doctors turned to genetics for explanations but found no conclusive results. Here are some of their findings-

  • Those with trisomy 21 seem to be particularly vulnerable to lung disease
  • Most patients diagnosed with the condition were only 2 years old
  • Some of the patients diagnosed with the condition had macrophage activation syndrome in the past
  • Some of the patients had well controlled sJIA when the LD presented
  • Some of the patients had hard-to-control disease when the LD presented

Correlation vs Causation

There is a very evident correlation with the rise of this lung disease and the rise of the widespread therapy used to treat the disease. However, correlation does not equal causation. Doctors are standing firm that the general practice should not change until more research has been completed. That doesn’t mean they aren’t worried about the trend that’s been witnessed. But the fact stands that this therapy has vastly changed the lives of the majority of patients living with sJIA for the better.

They explain that the rise of the lung disease could be a trend that is completely unrelated to the therapy. It could also be caused by a declining use of steroids and methotrexate. These drugs could have actually been preventing the lung disease. There are many potential links, and none of them have yet to be proven.

Regardless of the cause, it is important to know the symptoms of this disease. Surprisingly, respiratory symptoms with sJIA-LD are fairly mild. However, other symptoms that may present and should raise a red flag are abdominal pain, rash, finger clubbing, or lymphopenia. There is some evidence that adult patients with arthritis may also experience the same condition.

As researchers continue to work to learn more, it is important for patients to stay aware of their disease and potential symptoms they might be experiencing.

Hopefully we will know more soon.

You can read more about this research here.


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