Arthritic Kids Face Lung Problems

Systemic juvenile idiopathic arthritis patients can develop rare but deadly lung complications

/ Author:  / Reviewed by: Robert Carlson, M.D

(RxWiki News) Inflammatory arthritis isn't only for adults. Children can develop the condition too. This means they are not only faced with pain but also complications of living with an inflammatory disease.

Children with systemic juvenile idiopathic arthritis - also known as juvenile rheumatoid arthritis - may have a higher risk of serious lung problems than was previously thought, according to recent research.

In addition, more than half of patients in the small study had macrophage activation syndrome (MAS) - a potentially deadly complication of rheumatic diseases that occurs more frequently in patients with systemic juvenile idiopathic arthritis.

According to the study's authors, these risks may be caused by uncontrolled disease and exposure to certain drugs.

"Find treatment for your child's arthritis to prevent complications."

The research was conducted by Yukiko Kimura, MD, of Hackensack University Medical Center, and colleagues. The researchers compared a small group of juvenile arthritis patients with lung complications to a larger group of patients without such complications.

Systemic juvenile idiopathic arthritis is different from other types of juvenile idiopathic arthritis and is linked to fevers, rash and health problems in different organs.

The lung complications seen in the study included:

  • pulmonary arterial hypertension - high blood pressure in the arteries of the lungs
  • interstitial lung disease - scarring and damage of lung tissues
  • alveolar proteinosis - a disease in which a certain protein builds up in the air sacs of the lungs, which makes it hard to breath

Even though these complications are quite rare, they can be life threatening.

In the study, 16 patients (64 percent) had pulmonary arterial hypertension, seven patients (28 percent) had interstitial lung disease and five patients (20 percent) had alveolar proteinosis.

Dr. Kimura and colleagues found that patients with lung complications were more likely to have systemic features - or aspects of the disease affecting multiple parts of the body - compared to those without lung complications.

Of the patients with lung complications, 20 patients (80 percent) had MAS during the course of their disease and 15 patients (60 percent) had MAS when they were diagnosed with lung problems.

A total of 17 patients (68 percent) died within about 9 months of being diagnosed with lung complications.

In many cases, MAS occurs when the immune system speeds up to fight infection but does not slow down when the infection goes away. It is also believed that MAS may be triggered by certain medications.

According to the study's results, patients with lung complications were more likely to have been exposed to a variety of drugs, including:

  • IL-1 inhibitors
  • Actemra (tocilizumab)
  • Remicade (infliximab)
  • corticosteroids
  • intravenous immunoglobulin
  • cyclosporine
  • cyclophosphamide, which is sold as Cytoxan, Endoxan and Neosar, among others

A total of 17 patients (68 percent) were taking or recently stopped taking a biologic drug when symptoms of lung complications began.

Both Actemra and Remicade are types of biologic drugs.

In addition, 12 patients (48 percent) were taking an IL-1 inhibitor when lung symptoms started. The most common IL-1 inhibitor was Kineret (anakinra).

"Pulmonary arterial hypertension, alveolar proteinosis and interstitial lung disease are under-recognized complications of systemic juvenile idiopathic arthritis which are frequently fatal. These may be the results of severe uncontrolled systemic disease activity, and may be influenced by medication exposure," the authors concluded.

The research included 389 juvenile arthritis patients without lung complications and only 25 patients with lung complications. Due to the small size of the study, more research is needed to confirm these results in a larger population.

Several of the study's authors reported ties to the pharmaceutical industry.

The study was published November 8 in Arthritis Care & Research, a journal of the American College of Rheumatology.

Reviewed by: 
Review Date: 
December 2, 2012
Last Updated:
April 11, 2013