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.

Atypical Clinical Presentation of Rapidly Progressing Femoral Head Avascular Necrosis.

A 51-year-old man presented to an urgent care facility 2 weeks after onset of left lateral hip and buttock pain. Radiographs were noncontributory. An orthopaedist referred him to physical therapy, but due to the worsening clinical presentation, he was referred back to the orthopaedist for additional evaluation. Magnetic resonance imaging was ordered and demonstrated abnormal femoral head and acetabular contour, extensive bone marrow edema, and a complex joint effusion. He was diagnosed with femoral head avascular necrosis. J Orthop Sports Phys Ther 2017;47(3):217. doi:10.2519/jospt.2017.6483.

Inferior vena cava size is not associated with shock following injury.

BACKGROUND: The inferior vena cava (IVC) collapses with shock but may also be collapsed in volume-depleted patients in the absence of shock. The speed and availability of computed tomography (CT) make IVC measurement an attractive diagnostic modality for shock. The purpose of this study was to determine if IVC size following injury is associated with shock. METHODS: Retrospective data were collected on 272 trauma patients admitted to an adult trauma center from January 1 to December 31, 2012. Patients who met the highest-level activation criteria and underwent an abdominal CT scan during their initial resuscitation were included. All images were reviewed by two attending radiologists, and concordance was assessed using the Pearson correlation coefficient. The transverse (T) and anteroposterior (AP) diameters of the IVC were measured to calculate a T/AP ratio. Analysis of variance and χ were used to assess for a relationship between this ratio and various indices of shock. RESULTS: The mean (SD) age of the study cohort was 50 (21) years, mean (SD) Injury Severity Score (ISS) was 14 (9), 74% were male, and 96% sustained blunt trauma. The overall mean (SD) T/AP ratio was 1.81 (0.68). Patients with a shock index greater than 0.7 were significantly younger (43 [20] years vs. 55 [21] years, p < 0.0001), had a significantly lower mean arterial pressure (88 [15] mm Hg vs. 103 [18] mm Hg, p < 0.0001), and were more likely to be intubated (56% vs. 24%, p < 0.0001). However, IVC T/AP ratio was not significantly different among the cohort. Similarly, there was no association between IVC size and the need for urgent operation, angiography, emergent transfusion, hospital length of stay, or mortality. CONCLUSION: The static degree of IVC collapse is not associated with shock following injury. Therefore, measurement of IVC size by CT scan for patients with a T/AP ratio between 1 and 3.5 is not clinically relevant and cannot be used to predict mortality, shock, or impending shock. LEVEL OF EVIDENCE: Diagnostic test, level III.

Migrated Essure permanent birth control device: sonographic findings.

We report a case of a migrated Essure permanent birth control device. The correct diagnosis was made on conventional two-dimensional and three-dimensional pelvic sonography 7 years after placement of the device when the patient presented with persistent right-sided pain. The 3-month post placement hysterosalpingogram had shown an appropriately occluded right fallopian tube but had overlooked the abnormal position of the right Essure device, which was too proximal and extending slightly in the uterine cavity.

Average chest wall thickness at two anatomic locations in trauma patients.

BACKGROUND: Needle thoracostomy is the emergent treatment for tension pneumothorax. This procedure is commonly done using a 4.5cm catheter, and the optimal site for chest wall puncture is controversial. We hypothesize that needle thoracostomy cannot be performed using this catheter length irrespective of the site chosen in either gender. METHODS: A retrospective review of all chest computed tomography (CT) scans obtained on trauma patients from January 1, 2011 to December 31, 2011 was performed. Patients aged 18 and 80 years were included and patients whose chest wall thickness exceeded the boundary of the images acquired were excluded. Chest wall thickness was measured at the 2nd intercostal (ICS), midclavicular line (MCL) and the 5th ICS, anterior axillary line (AAL). Injury severity score (ISS), chest wall thickness, and body mass index (BMI) were analyzed. RESULTS: 201 patients were included, 54% male. Average (SD) BMI was 26 (7)kg/m(2). The average chest wall thickness in the overall cohort was 4.08 (1.4)cm at the 2nd ICS/MCL and 4.55 (1.7)cm at the 5th ICS/AAL. 29% of the overall cohort (27 male and 32 female) had a chest wall thickness greater than 4.5cm at the 2nd ICS/MCL and 45% (54 male and 36 female) had a chest wall thickness greater than 4.5cm at the 5th ICS/AAL. There was no significant interaction between gender and chest wall thickness at either site. BMI was positively associated with chest wall thickness at both the 2nd and 5th ICS/AAL. CONCLUSION: A 4.5cm catheter is inadequate for needle thoracostomy in most patients regardless of puncture site or gender.

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.

Correlation of magnetic resonance imaging to arthroscopic findings of stability in juvenile osteochondritis dissecans.

PURPOSE: To determine the ability of magnetic resonance imaging (MRI) to characterize the stability of osteochondritis dissecans (OCD) fragments in juveniles. METHODS: Twenty-eight consecutive patients underwent surgery for OCD between 2004 and 2008. Of these, 23 patients had adequate preoperative imaging. There were 14 boys and 9 girls with a mean age of 12.9 years. Of the 23 lesions, 21 were located in the knee and 2 were located in the talus. On the basis of MRI, a single radiologist (1) indicated the presence or absence of 4 established magnetic resonance signs of instability, (2) classified each lesion according to a staging system for OCD stability, and (3) described the lesion as stable or unstable. These findings were compared with the arthroscopic findings. Arthroscopy was considered the gold standard for diagnosing fragment stability. RESULTS: Of the OCD lesions, 13 were found to be stable and 10 were found to be unstable. The final MRI impression was unstable in 21 patients and stable in 2 patients. This yielded a sensitivity of 100% and a specificity of 15% for diagnosing fragment instability. When 2 or more criteria were present, the specificity of MRI to classify lesion instability improved to 92%. The sensitivity, however, dropped to 50%. Concordance between arthroscopic stage and MRI stage was 30% (7 of 23). CONCLUSIONS: MRI predicted 21 of 23 lesions to be unstable, whereas arthroscopy found only 10 of these 23 lesions to be unstable. The most common pattern of false-positive findings involved lesions with an area of high signal intensity at the bone-fragment interface. MRI should not be used in isolation to determine lesion instability in young patients with juvenile OCD.

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.

MRI for assessing erosion and joint space narrowing in inflammatory arthropathies.

The superior soft tissue contrast and multiplanar capability of magnetic resonance imaging has contributed to earlier diagnosis and implementation of effective treatment for a variety of arthropathies. Owing to overlapping clinical signs and symptoms, MRI plays a role in delineating the features and stages of these conditions. With the advent of disease-modifying therapies, it is important to diagnose inflammatory arthropathy as early as possible. In this chapter, we discuss the pathophysiology of bone erosion and joint space narrowing, as well as the role of MRI in the imaging of the seropositive and seronegative inflammatory arthropathies.

Magnetic resonance imaging of the idiopathic inflammatory myopathies: structural and clinical aspects.

Idiopathic inflammatory myopathies are chronic diseases clinically characterized by symmetrical proximal muscle weakness. MRI has assumed a major role in the evaluation and management of these conditions. It is sensitive to the presence of inflammation and edema, especially with incorporation of fat suppression sequences, so it is a useful tool for establishing an early diagnosis, for evaluating the extent and number of lesions, and for determining the right site for biopsy. The noninvasive nature of the procedure makes it ideal for serial studies to evaluate response to treatment. Whole-body MRI can scan a large volume of muscles without prolonged acquisition time and has the potential to identify previously unsuspected sites of involvement. MRI is also an excellent technique for identifying areas of fatty infiltration within the muscles, which usually occurs in the late stages of inflammatory myopathies. In summary, MRI has revolutionized the way muscular diseases are diagnosed and treated.