3D MRI of the Rheumatic Diseases.

Magnetic resonance imaging (MRI) is commonly used to evaluate musculoskeletal pathologies due to its high spatial resolution and excellent tissue contrast. The diagnosis of rheumatic diseases can often be challenging. Investigation with conventional two-dimensional MRI is helpful for diagnosis and monitoring treatment. In the past few years, three-dimensional (3D) MRI has been more commonly used to assess joint pathologies including inflammatory and rheumatic diseases. This review discusses the techniques and protocols of 3D MRI and its diagnostic yield in the assessment of rheumatic diseases, along with different examples.

What's in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis.

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. METHODS: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. RESULTS: Of 31 patients included, 67.7% were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48% of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70%. Autoimmune disorders were found in 9.6%. In total, 13 patients survived (44.8%). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. CONCLUSION: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.

Cross-sectional imaging of adult crystal and inflammatory arthropathies.

This article highlights the key aspects and current perspectives of the role of cross-sectional imaging in adult crystal and inflammatory arthropathies in adults, briefly discussing CT, and particularly focusing on MRI and US imaging as it supplements the conventional radiography. The role of conventional and advanced MR imaging techniques and imaging findings in this domain is discussed and illustrated with case examples. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article contains images and data, which were collected from patients as a part of a retrospective IRB from the institutional teaching files and informed consent was waived.

Predicting Rheumatoid Arthritis Development Using Hand Ultrasound and Machine Learning-A Two-Year Follow-Up Cohort Study.

BACKGROUND: The early diagnosis and treatment of rheumatoid arthritis (RA) are essential to prevent joint damage and enhance patient outcomes. Diagnosing RA in its early stages is challenging due to the nonspecific and variable clinical signs and symptoms. Our study aimed to identify the most predictive features of hand ultrasound (US) for RA development and assess the performance of machine learning models in diagnosing preclinical RA. METHODS: We conducted a prospective cohort study with 326 adults who had experienced hand joint pain for less than 12 months and no clinical arthritis. We assessed the participants clinically and via hand US at baseline and followed them for 24 months. Clinical progression to RA was defined according to the ACR/EULAR criteria. Regression modeling and machine learning approaches were used to analyze the predictive US features. RESULTS: Of the 326 participants (45.10 ± 11.37 years/83% female), 123 (37.7%) developed clinical RA during follow-up. At baseline, 84.6% of the progressors had US synovitis, whereas 16.3% of the non-progressors did (p < 0.0001). Only 5.7% of the progressors had positive PD. Multivariate analysis revealed that the radiocarpal synovial thickness (OR = 39.8), PIP/MCP synovitis (OR = 68 and 39), and wrist effusion (OR = 12.56) on US significantly increased the odds of developing RA. ML confirmed these US features, along with the RF and anti-CCP levels, as the most important predictors of RA. CONCLUSIONS: Hand US can identify preclinical synovitis and determine the RA risk. The radiocarpal synovial thickness, PIP/MCP synovitis, wrist effusion, and RF and anti-CCP levels are associated with RA development.

Spectrum of Hand Arthritis: Doppler Ultrasound, Diffusion-Weighted MR Imaging, and Perfusion MR Imaging Evaluation.

Early diagnosis of arthritis is of paramount importance to slow the progression of disease and joint destruction. Because of temporal dissemination of the clinical and laboratory manifestations of the inflammatory arthritis and overlap of the findings, diagnosis can be challenging in early stages of the disease. This article highlights the utility of advanced cross-sectional imaging, including color-Doppler ultrasound, diffusion-weighted MR imaging, and perfusion MR imaging in the domain of arthropathy so that the reader can apply these principles and techniques in their practices for timely and accurate diagnosis and improved multidisciplinary communications for better management of such conditions.

Radiographic Findings of Inflammatory Arthritis and Mimics in the Hands.

Clinical presentation could be challenging in patients with arthralgia, and imaging plays an important role in the evaluation of these patients to make the diagnosis or narrow the differential diagnosis. Radiography of the hands is a commonly available imaging modality that can provide crucial information with regard to the pattern and pathology of the involved joints. It is important that radiologists and rheumatologists are familiar with the imaging findings of different rheumatic diseases to make the diagnosis in the early stages of disease to initiate treatment.

Three tesla and 3D multiparametric combined imaging evaluation of axial spondyloarthritis and pelvic enthesopathy.

PURPOSE: Axial spondyloarthritis (SpA) is a group of diseases with temporally disseminated symptoms and clinical signs, which render the diagnosis challenging. Laboratory and MRI findings are used in addition for confirming the diagnosis and evaluation of disease activity. The purpose of this study was to evaluate clinically suspected axial SpA to determine the technical success of a multiparametric and 3D rheumatology lumbosacral MR imaging (MRLI) protocol and to assess the disease distribution, inter-reader reliability, and impact on patient management. METHODS: A consecutive series of patients with clinical suspicion of axial SpA were included. Two rheumatologists recorded the clinical findings and disease activity on a confidence scale before and after MRLI. Two musculoskeletal (MSK) radiologists read the imaging data including enthesitis, arthritis, osseous lesions, ADC values, and enhancement. Prevalence-adjusted and bias-adjusted kappa (PABAK), ICC and Fisher exact test were calculated. RESULTS: There were 41 patients including 31 females and 10 males with ages of 41 ± 10 and 41 ± 12 (mean ± SD), respectively. The spine T2W imaging received the highest quality scores followed by whole abdomen-pelvis 3D-T2W imaging, 3D-CEMR (contrast-enhanced MRI), and DWI. On spine imaging, acute and chronic lesions of lumbar spine and sacroiliac joints were seen in 4/41, 18/41 and 6/41, and 27/41 of the patients, respectively. Several additional enthesopathy lesions were seen on the whole abdomen-pelvis 3D sequence. ADC value of bone lesions was different 0.95 ± 0.23 (mean ± SD) than normal bone (0.20 ± 0.1). PABAK for acute and chronic findings ranged 0.70-1.0 and 0.41-0.51, respectively. Imaging changed the diagnosis in 17 of 41 patients. No association was noted with respect to treatment change (p = 1) or clinical response (p = 0.2). CONCLUSION: Multiparametric lumbosacral MR imaging is a technically successful modality to identify multiple spinal and additional extraspinal sites of involvement in SpA, which are helpful in establishing the diagnosis of axial SpA. Larger patient population study is warranted to evaluate further impact on the treatment efficacy.

Serum zinc and its relation to bone mineral density in beta-thalassemic adolescents.

Trace elements have been considered to play critical roles in bone metabolism. This study aims at determining the serum zinc profile and its association with bone mineral density (BMD) abnormalities in thalassemic patients. In 131 transfusion-dependent beta-thalassemic patients, aged 10-20 yr, serum levels of zinc were measured by flame atomic absorption spectrophotometry (F-AAS). BMD values at the lumbar (L1-L4) and femoral neck were determined by dual X-ray absorptiometry (DXA). Dietary zinc intake and daily consumption of calcium were evaluated by a food-frequency questionnaire. Low serum zinc was found in 84.8% (in 44.7% severely low). Below -2 BMD Z-scores were observed in 68.7% and 17.6% of the patients at the lumbar and femoral regions, respectively. Female patients with severe zinc deficiency had lower lumbar BMD Z-scores in comparison to the other females (-3.26 vs -2.54). Serum zinc in females with femoral BMD Z-scores < - 2 was significantly lower by 16.4 microg/dL than other females. Our study suggests that serum levels of zinc can be lowered in the thalassemic patients and partly affect the BMD.

Bipolar supernumerary renal artery.

The variations of renal arteries are considered critical issues that surgeons should have thorough envision and appreciation of the condition. Variations of these vessels may influences urological, renal transplantation and laparoscopic surgeries. We present a case of bilateral accessory renal artery with a striking pre-hilar branching pattern encountered upon digital subtraction angiography (DSA) for imaging of the renal arteries of a healthy 30-year-old man, renal transplant donor. The right kidney received two renal arteries from the aorta including a main hilar and one lower polar. However, the left accessory artery while originated from the aorta, simultaneously, supplied both upper and lower renal poles following its pre-hilar division that replaced upper/apical and lower segmental arteries of the single main renal artery, respectively. The left main renal artery divided into two anterior and posterior segmental arteries. Whether this should be categorized either as an accessory hilar artery or a unique variant of renal arterial supply, the so-called bipolar supernumerary renal artery, is a matter of debate. We discuss possible embryologic origin and clinical aspects of accessory renal artery.