Preliminary Definitions for Sacroiliac Joint Pathologies in the OMERACT Juvenile Idiopathic Arthritis MRI Score (OMERACT JAMRIS-SIJ).

Objective: To develop definitions for the assessment of MRI pathologies of the sacroiliac joints (SIJ) in juvenile idiopathic arthritis (JIA). Methods: An OMERACT consensus-driven methodology consisting of iterative surveys and focus group meetings within an international group of rheumatologists and radiologists. Results: Two domains, inflammation and structural, were identified. Definitions for bone marrow edema, joint space inflammation, capsulitis and enthesitis were derived for joint inflammation; sclerosis, erosion, fatty lesion and ankylosis were defined for assessing structural joint changes. Conclusion: Preliminary consensus-driven definitions for inflammation and structural elements have been derived, underpinning the ongoing development of the JAMRIS-SIJ score

Standardizing Terminology and Assessment for Orofacial Conditions in Juvenile Idiopathic Arthritis : International, Multidisciplinary Consensus-based Recommendations

Objective. To propose multidisciplinary, consensus-based, standardization of operational terminology and method of assessment for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). Methods. Using a sequential expert group–defined terminology and methods-of-assessment approach by (1) establishment of task force, (2) item generation, (3) working group consensus, (4) external expert content validity testing, and (5) multidisciplinary group of experts final Delphi survey consensus. Results. Seven standardized operational terms were defined: TMJ arthritis, TMJ involvement, TMJ arthritis management, dentofacial deformity, TMJ deformity, TMJ symptoms, and TMJ dysfunction. Conclusion. Definition of 7 operational standardized terms provides an optimal platform for communication across healthcare providers involved in JIA-TMJ arthritis management.publishersversionPeer reviewe

Reliability of the Preliminary OMERACT Juvenile Idiopathic Arthritis MRI Score (OMERACT JAMRIS-SIJ)

This study reports the reliability of the juvenile idiopathic arthritis magnetic resonance imaging scoring system (JAMRIS-SIJ). The study comprised of eight raters—two rheumatologists and six radiologists—and 30 coronal T1 and Short-Tau Inversion Recovery (STIR) MRI scans of patients with enthesitis-related juvenile spondylarthritis. The median age of patients was 15 years with a mean disease duration of 5 years and 22 (73.3%) of the sample were boys. The inter-rater agreement of scores for each of the JAMRIS-SIJ items was calculated using a two-way random effect, absolute agreement, and single rater intraclass correlation coefficient (ICC 2.1). The ICC was interpreted together with kurtosis, since the ICC is also affected by the distribution of scores in the sample. The eight-rater, single measure inter-rater ICC (and kurtosis) values for JAMRIS-SIJ inflammation and damage components were the following: bone marrow edema (BME), 0.76 (1.2); joint space inflammation, 0.60 (1.8); capsulitis, 0.58 (9.2); enthesitis, 0.20 (0.1); ankylosis, 0.89 (35); sclerosis, 0.53 (4.6); erosion, 0.50 (6.5); fat lesion, 0.40 (21); backfill, 0.38 (38). The inter-rater reliability for BME and ankylosis scores was good and met the a priori set ICC threshold, whereas for the other items it was variable and below the selected threshold. Future directives should focus on refinement of the scores, definitions, and methods of interpretation prior to validation of the JAMRIS-SIJ through the assessment of its measurement properties

Temporomandibular joint atlas for detection and grading of juvenile idiopathic arthritis involvement by magnetic resonance imaging

Contrast-enhanced magnetic resonance imaging (MRI) is considered the diagnostic standard for identifying involvement of the temporomandibular joint by juvenile idiopathic arthritis. Early or active arthritis is shown as bone marrow oedema, joint effusion, synovial thickening and increased joint enhancement. Subsequent joint damage includes characteristic deformity of the mandibular condyle, bone erosion, disk abnormalities and short mandibular ramus due to impaired growth. In this pictorial essay, we illustrate normal MRI findings and growth-related changes of the temporomandibular joint in children. The rationale and practical application of semiquantitative MRI assessment of joint inflammation and damage are discussed and presented. This atlas can serve as a reference for grading temporomandibular joint arthritis according to the scoring systems proposed by working groups of OMERACT (Outcome Measures in Rheumatology and Clinical Trials) and the EuroTMjoint research network. Systematic assessment of the level of inflammation, degree of osteochondral deformation, and growth of the mandibular ramus by MRI may aid in monitoring the course of temporomandibular joint arthritis and evaluating treatment options

Assessing the Reliability of the OMERACT Juvenile Idiopathic Arthritis Magnetic Resonance Scoring System for Temporomandibular Joints (JAMRIS-TMJ)

Contrast-enhanced magnetic resonance imaging (MRI) remains the most comprehensive modality to assess juvenile idiopathic arthritis (JIA)-related inflammation and osteochondral damage in the temporomandibular joints (TMJ). This study tested the reliability of a new JIA MRI scoring system for TMJ (JAMRIS-TMJ) and the impact of variations in calibration and reader specialty. Thirty-one MRI exams of bilateral TMJs were scored independently using the JAMRIS-TMJ by 20 readers consisting of radiologists and non-radiologist clinicians in three reading groups, with or without a calibrating atlas and/or tutorial. The inter-reader reliability in the multidisciplinary cohort assessed by the generalizability coefficient was 0.61–0.67 for the inflammatory and 0.66–0.74 for the damage domain. The atlas and tutorial did not improve agreement within radiologists, but improved the agreement between radiologist and non-radiologist groups. Agreements between different calibration levels were 0.02 to 0.08 lower by the generalizability coefficient compared to agreement within calibration levels; agreement between specialty groups was 0.04 to 0.10 lower than within specialty groups. Averaging two radiologists raised the reliability above 0.8 for both domains. Therefore, the reliability of JAMRIS-TMJ was moderate-to-good depending on the presence of specialty and calibration differences. The atlas and tutorial are necessary to improve reliability when the reader cohort consists of multiple specialties

Towards Establishing and Validating an MRI Scoring System for Assessment and Interval Monitoring of Temporomandibular Joints in Juvenile Idiopathic Arthritis

Background: Contrast enhanced magnetic resonance imaging (MRI) is the most comprehensive diagnostic tool for the evaluation of temporomandibular joints (TMJ) in juvenile idiopathic arthritis (JIA). However, its acquisition and interpretation remain variable across centres. Purpose: 1) To construct a scoring system for the MRI-observable manifestations of JIA in the TMJ (JAMRIS-TMJ), 2) determine and validate its importance weights, and 3) test its reliability. Methods: First, an inter-reader reliability study with 21 cases and 7 readers was done to test the items of three existing TMJ MRI scoring systems. Then, a Delphi and nominal group technique process was used to construct a consensus scoring system (JAMRIS-TMJ) based on the reliability results and expert opinion. An imaging atlas and protocol were developed through subsequent consensus meetings. Next, the relative weights of the JAMRIS-TMJ were determined among experts using an adaptive, partial-profile discrete choice experiment (DCE). Group-averaged weights were validated using a series of 14 image vignettes. Finally, the reliability of JAMRIS-TMJ were tested in a 20-reader reliability study using 31 TMJ MRI exams across different levels of calibration and differences in reader specialty. Results: Consensus process driven by expert opinion and reliability data yielded a scoring system containing 5 inflammatory and 3 damage items in respective domains. DCE showed a homogenous group-level preference on weights, with items ranging from 9% to 49% of domain score. Vignette ranking exercise showed high correlation of vignette ranks between the partial profile, DCE-weighted method and the image-based ranking method, with median Spearman’s ρ of >0.90 for the two domains. Generalizability (G-) coefficients for domain score reliability were 0.61-0.67 for the inflammatory, and 0.66-0.74 for the damage domain, depending on the level of calibration. Atlas and tutorial did not affect agreement within radiologists but improved agreement between radiologists and non-radiologist clinicians. Reliability by G-coefficient reached >0.8 when taking the average of two reads. Conclusion: A multi-center, multi-specialty effort has led to the proposal of JAMRIS-TMJ, which demonstrated construct validity and moderate-to-good reliability.Ph.D

Invasive intravascular aspergillosis leading to myocardial infarction

Cardiac dissemination of invasive aspergillosis is a rare, often a fatal consequence of systemic mycosis. We present a heart transplant patient who succumbed to drug resistant aspergillus pneumonia. Myocardial ischemic damage from aspergillus vasculitis was likely the final event. Keywords: Invasive aspergillosis, Myocardial infarction, Heart transplant, Opportunistic infections, Vasculitis, Voriconazol

Assessing the Reliability of the OMERACT Juvenile Idiopathic Arthritis Magnetic Resonance Scoring System for Temporomandibular Joints (JAMRIS-TMJ)

Contrast-enhanced magnetic resonance imaging (MRI) remains the most comprehensive modality to assess juvenile idiopathic arthritis (JIA)-related inflammation and osteochondral damage in the temporomandibular joints (TMJ). This study tested the reliability of a new JIA MRI scoring system for TMJ (JAMRIS-TMJ) and the impact of variations in calibration and reader specialty. Thirty-one MRI exams of bilateral TMJs were scored independently using the JAMRIS-TMJ by 20 readers consisting of radiologists and non-radiologist clinicians in three reading groups, with or without a calibrating atlas and/or tutorial. The inter-reader reliability in the multidisciplinary cohort assessed by the generalizability coefficient was 0.61-0.67 for the inflammatory and 0.66-0.74 for the damage domain. The atlas and tutorial did not improve agreement within radiologists, but improved the agreement between radiologist and non-radiologist groups. Agreements between different calibration levels were 0.02 to 0.08 lower by the generalizability coefficient compared to agreement within calibration levels; agreement between specialty groups was 0.04 to 0.10 lower than within specialty groups. Averaging two radiologists raised the reliability above 0.8 for both domains. Therefore, the reliability of JAMRIS-TMJ was moderate-to-good depending on the presence of specialty and calibration differences. The atlas and tutorial are necessary to improve reliability when the reader cohort consists of multiple specialties

Whole-body MRI Quantification for Assessment of Bone Lesions in Chronic Nonbacterial Osteomyelitis Patients Treated With Pamidronate: A Prevalence, Reproducibility, and Responsiveness Study

ObjectiveThe purpose of this study was (1) to assess the interreader reliability in detecting and scoring the inflammatory bone lesions in pediatric patients with chronic nonbacterial osteomyelitis (CNO) by using whole-body magnetic resonance imaging (WB-MRI), and (2) to evaluate the responsiveness of the MRI-detected CNO lesions to pamidronate therapy.MethodsEighty-eight WB-MRI examinations were independently reviewed and scored by 2 radiologists blinded to clinical details in 32 retrospectively enrolled pediatric patients with CNO. Inflammatory bone lesions, soft tissue abnormality, and bony structural changes were scored before and after pamidronate therapy. Lesion responsiveness was calculated by using standardized response mean and interreader reliability was assessed by k statistics.ResultsThere was good to excellent interreader agreement for the detection and quantification of bone lesions. After the first cycle of pamidronate in all 32 patients, 96 of the 279 lesions (34%; after excluding 108 lesions of hand and feet) resolved, whereas in a subset of 11 patients with 2 or more cycles, 76% of lesions resolved after the second cycle. Twenty-one (7.5%) lesions worsened and 46 (16.4%) new lesions developed after 1 cycle in all 32 patients. In these 11 patients, the number of worsened lesions reduced to 2 (2%) and new lesions to 14 (14.9%) after the second cycle as detected on MRI. Vertebral lesions had the highest response to treatment.ConclusionWB-MRI is a reliable tool for objective quantification and assessment of response to treatment of pediatric CNO bone lesions and could be used to monitor disease activity for clinical and research purposes.</jats:sec