Improvement in Disease Activity in Refractory Juvenile Dermatomyositis Following Abatacept Therapy.

OBJECTIVE: This open-label, 24-week study was conducted to evaluate the safety and efficacy of abatacept in patients with refractory juvenile dermatomyositis (DM). METHODS: Ten patients ≥7 years of age with moderate disease activity were enrolled in a 24-week study to examine the safety of subcutaneous abatacept and patient responses to the treatment. The primary endpoint was the International Myositis Assessment and Clinical Studies (IMACS) group Definition Of Improvement (DOI). Secondary endpoints included safety, changes in the core set activity measures (CSMs) of the IMACS group and the Pediatric Rheumatology International Trials Organization, and improvements in disease activity based on the American College of Rheumatology (ACR)/EULAR response criteria for juvenile DM. Radiologists blinded with regard to participant data assessed magnetic resonance images (MRIs) of patient thigh muscles. Interferon (IFN)-regulated gene score was performed on whole-blood RNA samples using a NanoString assay, and cytokines were assessed using a Luminex assay. RESULTS: Five patients achieved DOI at week 12, and 9 patients achieved DOI at week 24, including 2 patients with minimal, 4 patients with moderate, and 3 patients with major improvement by the 2016 ACR/EULAR response criteria for juvenile DM when patients were assessed using the CSMs of the IMACS Group. Improvements from baseline were seen in all CSMs at weeks 12 and 24, except in muscle enzymes. Daily glucocorticoid doses decreased from a mean of 16.7 mg at baseline to 10.2 mg at week 24 (P = 0.002). Average MRI muscle edema scores decreased from a mean baseline score of 5.3 to 2.3 at week 24 (P = 0.01). Six patients had down-trending IFN-regulated gene scores and galectin-9 expression at week 24. Decreases in IFN-regulated gene scores and in levels of interferon-γ-inducible protein 10kDa, galectin-9, and interleukin-2 correlated with improvements in disease activity and in muscle edema shown on MRI. Eleven grade 2 or 3 treatment-emergent adverse events were observed. CONCLUSION: This open-label study demonstrated that abatacept may be beneficial for patients with treatment-refractory juvenile DM.

Multimodality imaging of intrauterine devices with an emphasis on the emerging role of 3-dimensional ultrasound.

The intrauterine contraceptive device (IUD) is one of the most widely used reversible contraception methods throughout the world. With advancing technology, it has rapidly gained acceptance through its increased effectiveness and practicality compared with more invasive means such as laparoscopic tubal ligation. This pictorial essay will present the IUDs most commonly used today. It will illustrate both normal and abnormal positions of IUDs across all cross-sectional imaging modalities including 2-dimensional ultrasound, computed tomography, and magnetic resonance imaging, with a focus on the emerging role of 3-dimensional ultrasound as the modality of choice.

The unicornuate uterus and its variants: clinical presentation, imaging findings, and associated complications.

This article will describe the different variants of the unicornuate uterus, their clinical presentation and imaging findings, as well their associated complications. We will also review the associated renal anomalies. Patients' symptoms and their imaging findings will vary depending on the unicornuate subtype. Radiologic evaluation includes a combination of hysterosalpingography, sonography, and magnetic resonance imaging. Complications include obstetric ones related to the small uterine size and endometriosis and ectopic pregnancies when a cavitary rudimentary uterine horn is present. Radiologists should be familiar with all variants of the unicornuate uterus as well as their clinical presentation and associated imaging findings.

Multimodality imaging of the essure permanent birth control device: emphasis on commonly overlooked abnormalities.

OBJECTIVE: The purpose of this pictorial essay is to review the imaging findings of normally and abnormally positioned Essure permanent birth control devices across the different imaging techniques and to show common sites of device migration. CONCLUSION: The Essure device is easily visualized on all imaging techniques, and radiologists should be familiar with its appearance and be able to identify cases of device migration on ultrasound and hysterosalpingography that would suggest malfunction.

Intra-articular knee mass in a 51-year-old woman.

A 51-year-old woman presented with moderate knee pain refractory to conservative measures. Radiographs revealed a well-defined, but irregularly-shaped ovoid soft tissue density at the posterior superior aspect of the infrapatellar fat pad of Hoffa. On magnetic resonance imaging, the mass was mildly heterogeneous and had intermediate signal in the proton density series and mixed signal intensity on the T2-weighted images. A low-signal rim could be seen around a portion of the lesion. Arthroscopic resection was performed and a vascular stalk was encountered. The differential diagnosis included: ganglion cyst, meniscal cyst, intra-articular lipoma, villous proliferation of the synovial membrane, Hoffa disease, and intracapsular chondroma. Histologic examination revealed a fibrous capsule partially surrounding a benign lipomatous neoplasm containing an abundance of thin- and thick-walled blood vessels with periadventitial myxoid stroma. To our knowledge, this is the first reported case of an intra-articular angiomyxolipoma in the literature. At 8-month follow-up, the patient was asymptomatic with no sign of recurrence. We postulate a low recurrence rate based on the insidious growth rate and benign histological appearance of such lesions. To our knowledge, no other intra-articular lipomatous lesion of the knee has been removed solely with arthroscopic techniques. Further follow-up information is needed to better understand the natural course of these lesions.

Goals and objectives for an integrated, graduated curriculum in abdominal radiology.

The Residency Review Committee of the American Council of Graduate Medical Education has designated abdominal radiology as one of the subspecialty areas required for radiology residency training. Because it spans both gastrointestinal and genitourinary organ systems and multiple modalities, a graduated, integrated abdominal radiology curriculum, which is based on the General Competencies, was developed.

Physeal changes and range-of-motion differences in the dominant shoulders of skeletally immature baseball players.

The purpose of this study was to document range-of-motion differences and radiographic changes in the dominant shoulder of skeletally immature throwers and to determine how pain associated with throwing may relate to these changes. Seventy-nine male youth baseball players (aged 8-15 years) completed a questionnaire, a shoulder examination, and a series of radiographs to determine physeal changes and humeral retroversion. Radiographs were reviewed and interpreted by a blinded musculoskeletal radiologist. Measurement of proximal humeral physeal width revealed a significant increase on the dominant side for the entire group, in subjects with a history of symptoms during the current season, and in subjects who had never had symptoms. Visual radiographic changes were commonly found in subjects with a history of pain (16/26 [62%]) as well as in those subjects without symptoms (29/53 [55%]). Subjects had increased external rotation of the dominant arm as compared with the nondominant arm, and this pattern increased in magnitude as the throwers aged. Range-of-motion and radiographic asymmetry of the shoulders is common, is often asymptomatic, and may represent adaptive changes in this population.