P41 Neutrophils from children with polyarticular and pauciarticular JRA show distinct gene expression profiles

Abstract

Juvenile rheumatoid arthritis (JRA), now termed juvenile idiopathic arthritis (JIA), is a well-described group of arthritides in the pediatric population with known associations with airway compromise. Subclasses are based on associated symptoms and number of joints involved, namely pauciarticular, polyarticular, and systemic onset JRA. Cricoarytenoid arthritis (CA), as a cause of airway compromise, is rarely associated with JRA as compared to adult onset rheumatoid arthritis, however, there are reports of CA with JRA. Additionally, JRA is associated with other rheumatologic diseases, which include a reported case of Wegener's granulomatosis. Wegener's granulomatosis has a well-established association with subglottic stenosis. Described below is the case study of an 18 year-old African American female with a 5-year history of pauciarticular juvenile rheumatoid arthritis developed worsening shortness of breath, orthopnea, and stridor over a 1-month period. The patient had a firm circumferential subglottic mass obstructing approximately 80% of the lumen and requiring tracheostomy tube placement. Biopsies of the mass revealed only acute and chronic inflammation. There was no evidence of rheumatoid nodules, granulomas, acid fast, or fungal organisms. To our knowledge, this is the first case history reported of a patient with JRA and idiopathic subglottic stenosis; this potentially establishes an association between the two disease processes.