Clinical Characteristics of Cryopyrin-Associated Periodic Syndrome and Long-Term Real-World Efficacy and Tolerability of Canakinumab in Japan: Results of a Nationwide Survey.

OBJECTIVE: We assess the clinical characteristics of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and evaluate the real-world efficacy and safety of interleukin-1 (IL-1) inhibitors, primarily canakinumab. METHODS: Clinical information was collected retrospectively, and serum concentrations of canakinumab and cytokines were analyzed. RESULTS: A total of 101 patients were included, with 86 and 15 carrying heterozygous germline and somatic mosaic mutations, respectively. We identified 39 mutation types, and the common CAPS-associated symptoms corresponded with those in previous reports. Six patients (5.9% of all patients) died, with four of the deaths caused by CAPS-associated symptoms. Notably, 73.7% of patients (100%, 79.6%, and 44.4% of familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and chronic infantile neurological cutaneous articular syndrome/neonatal onset multisystem inflammatory disease, respectively) achieved complete remission with canakinumab, and early therapeutic intervention was associated with better auditory outcomes. In some patients, canakinumab treatment stabilized the progression of epiphysial overgrowth and improved height gain, visual acuity, and renal function. However, 23.7% of patients did not achieve inflammatory remission with crucial deterioration of organ damage, with two dying while receiving high-dose canakinumab treatment. Serological analysis of canakinumab and cytokine concentrations revealed that the poor response was not related to canakinumab shortage. Four inflammatory nonremitters developed inflammatory bowel disease (IBD)-unclassified during canakinumab treatment. Dual biologic therapy with canakinumab and anti-tumor necrosis factor-α agents was effective for IBD- and CAPS-associated symptoms not resolved by canakinumab monotherapy. CONCLUSION: This study provides one of the largest epidemiologic data sets for CAPS. Although early initiation of anti-IL-1 treatment with canakinumab is beneficial for improving disease prognosis, some patients do not achieve remission despite a high serum concentration of canakinumab. Moreover, IBD may develop in CAPS after canakinumab treatment.

Associations Among Tooth Loss, Periodontitis, and Carotid Intima-Media Thickness: the Nagahama Study.

AIMS: This study aimed to clarify the relationships among tooth loss, periodontal condition, and subclinical atherosclerosis from the aspect of intensity, extent, and duration of inflammation. METHODS: This cross-sectional study included 9,778 people from the Nagahama Study, a large-scale, general population-based study conducted in Japan. The number of teeth and periodontal status, including the attachment level (AL) and pocket depth (PD) of representative teeth from six regions, were evaluated by dentists. The maximum intima-media thickness (IMT) of the common carotid artery was used as an index of atherosclerosis. RESULTS: In the multivariate analysis adjusted for conventional risk factors, a large number of missing teeth (＜9 remaining teeth), which related to long-lasting inflammation indicative of the highest stage of periodontitis, was identified as an independent determinant of IMT in a general population (coefficient: 0.042; 95% confidence interval [CI]: 0.016 to 0.068). The presence of two or more regions with an AL ≥4 mm, which is indicative of the progressing, long-lasting stages of periodontal inflammation, was also independently associated with IMT (coefficient: 0.016; 95% CI: 0.004 to 0.028). On the contrary, PD, a measure of the early and reversible phases of periodontal inflammation, and loss of AL in the group without tooth loss were not significantly associated with IMT, because of the limited degree of accumulated periodontitis. CONCLUSION: The present results suggest that the association between periodontitis and atherosclerosis depends on the inflammation intensity, extent, and duration.

Esthetics of Orthodontic Appliances: Objective Evaluation by Spectrophotometry vs Subjective Evaluation Using the Visual Analog Scale Method.

AIM AND OBJECTIVE: In this study, we quantified the color of brackets and archwire appliances for an objective evaluation and investigated its relationship with subjective esthetic evaluation. MATERIALS AND METHODS: Five types of commercially available brackets (ceramic brackets C1, C2, and C3; plastic brackets P1 and P2) and three types of archwires (coated nickel-titanium archwires W1, W2, and W3) were used. The reflectance (%) and color (lightness: L*, hue: a*, b*) of each sample were quantified using a spectrophotometer (n = 5). Fifteen combinations of brackets and archwires were used. The esthetic evaluation was performed using the visual analog scale (VAS) method, and responses were obtained from 30 laypersons and 15 orthodontists. The mean VAS score was calculated, and the relationship between the reflectance and color of the bracket and archwire was discussed. RESULTS: The reflectance and L* of the brackets showed significantly higher values for C3 and C1 than for the others and lower values for P1 and P2. The reflectance and L* of the archwire showed significant differences among all samples. There was a high positive correlation between the reflectance and L*. There were statistically significant positive correlations between the layperson and orthodontist groups, between the VAS score and reflectivity, and between VAS score and L*. CONCLUSION: Our results showed that as the lightness and reflectance of the brackets and archwires increased, the subjective evaluation concerning their esthetic value was higher. CLINICAL SIGNIFICANCE: It is extremely difficult to evaluate esthetics despite the fact that patients' demands for esthetics have been increasing in recent years. If a method for evaluating esthetics is established, it should help in the development and selection of esthetic devices. The results of this study will facilitate the development of future study designs.

Accuracy of predicting the pharyngeal airway space on the cephalogram after mandibular setback surgery.

PURPOSE: The aim of the present study was to evaluate the accuracy of a formula predicting the change of pharyngeal airway space (PAS) on the cephalogram after mandibular setback surgery. PATIENTS AND METHODS: Twenty-nine female adults with mandibular prognathism underwent surgery by bilateral sagittal split ramus osteotomy. The postoperative anteroposterior (A-P) PAS dimension was calculated from the change of C3-Me (distance between the anteroinferior point of the third cervical vertebra and menton) using 2 cephalograms taken before treatment and at 1 year after surgery. The regression equation used was y = -21.105 + 0.402x (y: PAS; x: C3-Me; r = 0.854). RESULTS: The predicted and measured values were nearly same on average, and the difference between both ranged within +/-1.5 mm for each case individually. CONCLUSION: This study suggested that the postoperative A-P PAS dimension can be predicted from the change in C3-Me, matching the amount of the mandibular setback.