Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis

Area of Science:

• Pediatric Rheumatology
• Infectious Diseases
• Clinical Immunology

Background:

• Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease in children.
• Refractory JIA often requires intensive immunosuppressive therapies, including biological disease-modifying antirheumatic drugs (DMARDs).
• These treatments increase the risk of severe infections and mortality in pediatric patients.

Observation:

• A study reviewed four pediatric deaths related to JIA between 1994 and 2013.
• Three deaths resulted from severe bacterial sepsis (coagulase-negative Staphylococcus, α-hemolytic Streptococcus) associated with central venous catheters.
• One death was attributed to disseminated adenovirus and Epstein-Barr virus infection.

Findings:

• All four deceased children had active, refractory JIA.
• They were undergoing long-term treatment with multiple conventional and biological DMARDs.
• Two patients died while on high-dose corticosteroids, methotrexate, and anti-tumor necrosis factor-α agents; two died during hematopoietic stem cell transplantation.

Implications:

• Severe infections pose a significant mortality risk in children with refractory JIA.
• Close monitoring for infections is crucial for children with JIA on immunosuppressive therapy.
• Accurate reporting of severe infections and deaths is vital for disease surveillance and improved patient outcomes.