Search

Kawasaki disease (KD) is associated with expression and methylation of Fc gamma receptor genes. We characterized immunoglobulin A (IgA), IgE, IgG, and IgM receptor expression levels in KD. Fc receptor expression levels were characterized using GeneChip Human Transcriptome Array 2.0 (HTA 2.0) with 18 KD patients, 18 non-febrile controls, and 18 febrile controls. Another 48 control individuals and 46 patients with KD were measured using pyrosequencing for the methylation levels. The mRNA expression levels of FCER1A and FCER2 were significantly lower in KD patients than in non-febrile controls and then rose following treatments with intravenous immunoglobulin (IVIG). Expression levels of FCER1G increased compared to the non-febrile subjects and then subsided after IVIG. FCER1A methylation was significantly lower among KD patients and even lower in KD patients with IVIG resistance. HTA analysis revealed higher mRNA levels of FCAR, FCGR1C, and FCGR2A in KD patients. FCMR mRNA expression levels were significantly lower in KD patients. FCMR expression levels rose after IVIG treatment. After IVIG, FCGR1A, B, and C decreased even lower than the febrile controls. This is the first study indicating that IgA, IgE, IgG, and IgM receptors are associated with KD. We highlighted potential biomarkers related to Fc receptors and their regulation.

Background Although the sternoclavicular joint (SCJ) may be involved in ankylosing spondylitis, rheumatic arthritis, and Behçet’s disease and participates in the systemic inflammatory process of arthritis, it is often neglected during routine rheumatologic clinical examinations. To the best of our knowledge, this is the first study to report etanercept treatment in juvenile idiopathic arthritis (JIA) with SCJ involvement. Case presentation In this study, we describe an unusual case of a child with juvenile idiopathic arthritis with an initial presentation of sternoclavicular mass. The patient (age, 14 years 10 months) presented with an insidious onset atraumatic swelling of the left SCJ and complained of right hip and bilateral ankle tenderness without an apparent cause. Initial ultrasonography indicated a heterogeneous mass in the left SCJ, while computed tomography identified mild swelling of the left SCJ with a thickened synovial lining, mild bone erosion, and some turbid fluid. The patient ultimately underwent left SCJ arthrotomy, during which tapping of the SCJ revealed 2 cc of yellowish fluid, inflammation and necrosis of tissues within the SCJ. A clear yellow joint fluid was aspirated, and testing revealed a negative culture result. The patient was diagnosed with JIA. The joint tenderness improved and erythrocyte sedimentation rate decreased after administering anti-tumor necrosis factor etanercept. An additional ultrasonography demonstrated that the initial imaging findings have been resolved. At the end of a 2-year follow-up period, the patient was completely symptom-free. Conclusions JIA with SCJ involvement is an uncommon presentation in adolescents. Etanercept may be a beneficial treatment for SCJ involvement in patients with JIA. The upper limbs showed no signs of limited range of motion during the follow-up period. Further studies are warranted to elucidate the efficacy of etanercept in JIA with sternoclavicular joint involvement.

Background The etiology of Kawasaki disease (KD) is still unknown; perinatal factors may have role with few studies. This study was aim to survey the perinatal factors and clinical outcome of KD, including coronary artery lesion (CAL) formation and intravenous immunoglobulin (IVIG) treatment response. Methods We enrolled a total of 185 KD patient–caregiver dyads in this study using questionnaires. The questionnaire included two categories: children’s characteristics, which consisted of age at disease onset, gender, gestational age at delivery, birth body weight, delivery methods, and breastfeeding status, and caregivers’ characteristics, which consisted of parents or not, education levels, maternal age at giving birth, total number of offspring, and family income. We analyzed the association of these factors with CAL formation and IVIG treatment response of KD. Results KD patients with CAL formation had a higher maternal age than non-CAL patients (32.49 ± 3.42 vs. 31.01 ± 3.92 years, p = 0.016). We also found that maternal age ≥ 32 years group had a higher rate of having KD patients with CAL (39/81 vs. 24/74, odds ratio 1.935, 95% confidence interval [1.007, 3.718], p = 0.047). The maternal age ≥ 35 years group had a higher rate of having KD patients with IVIG resistance (6/31 vs. 6/116, odds ratio 4.400, 95% confidence interval [1.309, 14.786], p = 0.01). There was no significant difference in either CAL formation or IVIG resistance in KD with regard to patient’s age at disease onset, gestational age, birth body weight, delivery methods, breastfeeding, caregiver type, caregivers’ education level, total number of offspring, or family income (p > 0.05). Conclusions This study is the first to report that maternal age is significantly associated with CAL formation and IVIG resistance in KD. We hypothesize that a maternal age less than 32 years would benefit KD offspring.