Long Term Safety and Efficacy of Etanercept in Juvenile Idiopathic Arthritis in a Single Center

OBJECTIVE: Our aim was to investigate the long term safety and efficacy of etanercept in children with juvenile idiopathic arthritis (JIA). METHODS: The study subjects were the 90 JIA patients treated with etanercept in the Department of Pediatrics, Hallym University Medical Center between January 2004 and December 2017. We retrospectively reviewed their medical records for age at diagnosis, duration of etanercept treatment, number of active joints, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and adverse events during treatment. RESULTS: Among the 90 patients, 38 (42.0%) were male and 52 (58.0%) were female; 15 (16.7%) had systemic onset, 41 (45.6%) had extended oligoarticular, 14 (15.6%) had rheumatoid factor-positive polyarticular, 18 (20.0%) had rheumatoid factor-negative polyarticular, and 2 (2.1%) had enthesitis-related arthritis. The median age at the start of etanercept treatment was 9 years (range, 3~18 years), and the median duration of etanercept treatment was 6 years (range, 0.5~13 years). The median number of active joints decreased from 9 to 0 after 6 months of etanercept treatment. The median CRP and ESR were within normal range after 3 months of treatment. Six patients experienced recurrence, 9 switched to other medications and 3 discontinued etanercept. Of the 14 reported adverse events, 1 was serious, and there were no tuberculosis infections or malignancies. CONCLUSION: Long-term treatment with etanercept is efficacious and safe for children with JIA. However, those with the systemic onset subtype appear to have low drug survival rate compared to those with other types of JIA.

Atrial fibrillation without cardiac anomaly in a 9-year-old child

Atrial fibrillation (AF), the most common chronic arrhythmia in adults, is rarely reported in children. Moreover, most of the previously reported children with AF have comorbidities, such as structural heart diseases, rheumatic diseases, and thyroid diseases. This case report is about a healthy 9-year-old boy who was diagnosed with AF without cardiac anomaly. He visited the emergency department with chest pain and palpitation, lasting 2 hours. His electrocardiogram showed narrow-complex tachycardia, which led to the diagnosis of supraventricular tachycardia. The administration of adenosine revealed rapid irregular P waves. After electrical cardioversion, cardiac rhythm was converted to normal sinus rhythm. This case report suggests that when children with narrow-complex tachycardia visit the emergency department, the possibility of AF, in addition to supraventricular tachycardia, should be considered if the RR intervals are markedly irregular.

New Provisional Classification of Juvenile Idiopathic Arthritis Applying Rheumatoid Factor and Antinuclear Antibody

OBJECTIVE: Previous classification systems for juvenile idiopathic arthritis (JIA) were based on the number of joints involved and did not categorize homogenous disease entities. Therefore, JIA patients were reclassified retrospectively by applying rheumatoid factor (RF) and antinuclear antibody (ANA), which have been proven to constitute a homogenous disease entity. METHODS: The medical records of JIA patients were investigated retrospectively and reclassified into six categories using the new provisional classification. The nomenclature was based on Dr. Martini's proposal in the 23rd European Paediatric Rheumatology Congress (2016) at Genoa, Italy. New categories included systemic JIA (sJIA), RF-positive JIA (RF-JIA), early-onset ANA-positive JIA (eoANA-JIA), enthesitis/spondylitis-related JIA (ESR-JIA), “other JIA”, and “unclassified JIA”. RESULTS: Of a total of 262 JIA patients, 71 (27.1%) were reclassified as sJIA, 31 (11.8%) as RF-JIA, 22 (8.4%) as eoANA-JIA, 63 (24.0%) as ESR-JIA, 65 (24.8%) as “other JIA”, and 10 (3.8%) as “unclassified JIA”. A comparison of RF-JIA, eoANA-JIA, and ESR-JIA revealed significant differences in the gender ratio, age of disease onset, and the cumulative number and type of joints involved among the three groups. “Other JIA” comprised a significant proportion (24.8%) and warrants the need for further classification. The characteristics of the RF-positive patients were comparable to those of the anti-cyclic citrullinated peptide antibody-positive patients. The ANA positivity was lower (28.2%) than that in Western studies but showed similar clinical features. CONCLUSION: This is the first study applying RF and ANA to classify JIA without considering the joint counts. The six new categories include sJIA, RF-JIA, eoANA-JIA, ESR-JIA, “other JIA,” and “unclassified JIA”.