Standardized reporting of spine and sacroiliac joints in axial spondyloarthritis MRI: from the ESSR-Arthritis Subcommittee.

OBJECTIVES: Apply a modified Delphi-based approach and produce a practical, radiology-specific set of definitions for interpretation and standardization of the multiple MRI findings in axial spondyloarthritis (ax-SpA), specifically to aid the general radiologist with a musculoskeletal interest, working with gold standard basic MRI protocols. MATERIALS AND METHODS: We report the results of a modified Delphi-based consensus of 35 experts from 13 countries in the Arthritis Subcommittee of the European Society of Musculoskeletal Radiology (ESSR). Seventeen definitions were created (i.e., nine for the spine and eight for the sacroiliac joint) and two Delphi rounds were conducted on an electronic database, collated and revised by the project leader with agreement. Group leads were appointed for each definition following the first round. Final definitions included only those that reached a consensus > 80%; if > 50% agreed on exclusion consensus, definitions were excluded. Final results have been shared during the Arthritis meeting at the Annual ESSR Congress. RESULTS: Fourteen definitions, eight for the spine and six for the sacroiliac joint were agreed for standardized reporting. Andersson's, anterior corner sclerotic and costovertebral joint inflammatory lesions of the spine, with active and non-active erosions, and fat metaplasia of the sacroiliac joint reaching the highest consensus (≥ 95%). More than 50% of the experts agreed to exclude joint space inflammation in the sacroiliac joint and tissue backfill. Syndesmophytes reached 76% agreement. CONCLUSIONS: Agreed definitions by expert radiologists using a modified Delphi process, should allow standardized actionable radiology reports and clarity in reporting terminology of ax-SpA. CLINICAL RELEVANCE STATEMENT: The proposed definitions will support reporting from musculoskeletal and general radiologists working with gold-standard basic MRI, improve confidence in lesion assessment, and standardize terminology to provide actionable reports on MRI in patients with ax-SpA. KEY POINTS: Experts applied a modified Delphi method to optimize the definitions of MRI findings of ax-SpA. After two Delphi rounds and one in-person meeting, fourteen definitions reached the agreement threshold. These consensus-based definitions will aid in actionable reporting specifically for the general radiologist with a musculoskeletal interest.

The usefulness of ultrasound in predicting outcomes in patients with shoulder pain: a prospective observational study.

OBJECTIVES: Shoulder pain is common but current clinical classification has limited utility. We aimed to determine whether groups of ultrasound-based shoulder pathologies exist and to evaluate outcomes according to identified groups and individual pathologies. METHODS: Prospective study of a community-based cohort with shoulder pain referred for their first ultrasound scan at a single radiology unit, with subsequent routine clinical care. Patient-reported outcomes were collected at baseline, 2 weeks and 6 months; standardised ultrasound reporting was employed. Latent class analysis (LCA) identified ultrasound pathology-based groups. Multiple linear regression analysis explored associations between baseline pathologies, subsequent treatment and shoulder pain and disability index (SPADI). Short-term response to corticosteroid injections was investigated. RESULTS: Of 500 participants (mean age 53.6; 52% female), 330 completed follow-up. LCA identified 4 groups: bursitis with (33%) or without (27%) acromioclavicular joint degeneration, rotator cuff tear (21%), no bursitis/tear (19%). Total SPADI was higher at baseline for cuff tears (mean 55.1 vs 49.7-51.3; overall p= 0.005), but accounting for this, groups did not differ at 6 months (43.5 vs 38.5-40.5; p= 0.379). Baseline SPADI was the only predictor of 6-month SPADI retained by penalised modelling; neither LCA-derived US groups nor individual pathologies were selected. Response to baseline injection at week 2 did not differ between groups (mean SPADI 40.1-43.8; p= 0.423). CONCLUSION: Ultrasound-based classification (groups or individual pathologies) of shoulder pain did not predict medium-term outcomes using current treatments. The role of routine diagnostic ultrasound for shoulder pain needs consideration; it may be useful if evidence-based therapies for specific pathologies are established.

The Evolving Roles of MRI and Ultrasound in First-Line Imaging of Rotator Cuff Injuries.

Rotator cuff tears are the most likely source of shoulder pain in adults and may cause protracted disability. Management of rotator cuff tears is associated with considerable costs. Accurate diagnosis can guide surgical planning and help achieve a favorable clinical outcome. Although radiography remains the initial imaging test for shoulder injury, the roles of MRI and ultrasound (US) as first-line imaging after radiography are evolving. This article leverages current literature and the practical experience of subspecialty musculoskeletal radiologists from different institutions in describing a practical approach to imaging rotator cuff pathology. Both MRI and US are accurate for identifying rotator cuff tears, but each has advantages and shortcomings. As both modalities currently represent reasonable first-line approaches, considerable practice variation has evolved. Given the low cost of US, imagers should strive to optimize the quality of shoulder US examinations and to build referrer confidence in this modality. The roles of direct CT and MR arthrography as well as imaging evaluation of the postoperative rotator cuff are also considered. Through careful selection among the available imaging modalities and optimal performance and interpretation of such examinations, radiologists can positively contribute to the diagnosis and treatment of patients with rotator cuff injuries.

Muscle stiffness in rheumatoid arthritis is not altered or associated with muscle weakness: A shear wave elastography study.

Objectives: To investigate muscle stiffness and strength in rheumatoid arthritis patients compared to healthy controls.Methods: A sample of 80 RA patients from three discrete groups: 1 - newly diagnosed treatment-naïve RA (n = 29), 2 - active RA for at least 1 year (n = 18) and 3 - in remission RA for at least 1 year (n = 33), was compared to 40 healthy controls. Shear wave velocity (SWV) was measured using shear wave elastography as a surrogate for tissue stiffness in multiple muscles. All participants performed isometric grip strength, timed get-up-and-go test, 30-s chair stand test and isokinetic knee extension/flexion (60°/s). The difference in SWV amongst the groups was tested using one-way ANOVA, and the correlation between SWV and muscle strength results were calculated using Pearson's coefficients.Results: The mean age ± SD was 61.2 ± 12.8 for RA patients and 61.5 ± 10.5 years for controls. SWV was not significantly different amongst the groups on all muscles (p > .05). In comparison to controls, the new and active RA groups showed a significantly lower isokinetic strength by -29% (p = .013) and -28% (p = .040), fewer chair stands by -28% (p = .001) and -44% (p < .001), longer walking times by -25% (p = .025) and -30% (p = .001), respectively, and weaker grip strength by -45% for both (p < .001). The muscle strength in the remission RA groups was not significantly lower, except in the isokinetic knee strength (-21%; p = .027). The correlations between SWE and the muscle assessment results were weak and insignificant (r < 0.30; p > .05).Conclusion: Significant muscle weakness was demonstrated in patients with RA disease. However, muscle stiffness was normal and not associated with muscle strength.

The European Society of Skeletal Radiology Quiz 2018.

This article consists of a series of clinical cases presented during the ESSR Quiz session at the 25th Annual Meeting of the European Society of Musculoskeletal Radiology in Amsterdam 2018. The first section contains the clinical information and an initial set of images for each case. The second section reveals the answers along with additional imaging followed by a short discussion about the pathologic processes presented. This article provides self-assessment and at the same time refreshes the reader's knowledge about some common and not-so-common clinical conditions that a radiologist may encounter during clinical practice.

The effect of ageing on shear wave elastography muscle stiffness in adults.

BACKGROUND: Skeletal muscle undergoes structural changes with ageing which may alter its biomechanical properties. Shear wave elastography (SWE) may detect these changes by measuring muscle stiffness. AIMS: To investigate muscle stiffness in healthy young, middle-aged and elderly cohorts using SWE and correlate it with muscle strength and mass. METHODS: Shear wave velocity (SWV) was measured in the quadriceps, hamstrings and biceps brachii of 26 young (range 20-35 years), 21 middle-aged (40-55) and 30 elderly (77-94) volunteers. The participants performed several muscle tests to evaluate their strength. The One-way ANOVA was used to test the muscle stiffness differences between the groups and the Pearson's correlation coefficient to evaluate the relationship between SWV and muscle strength. RESULTS: The overall resting muscle SWV gradually decreased with age but was only significantly reduced in the elderly group (p < 0.001); with the exception of the vastus lateralis SWV where a significant difference was noted (p < 0.05) between young (1.77 m/s), middle-aged (1.64 m/s) and elderly (1.48 m/s). The elderly group had on average 16.5% lower muscle stiffness compared to the young. SWV significantly correlated with muscle mass (r = 0.316), walking time (r = - 0.560), number of chair stands (r = 0.522), handgrip strength (r = 0.436) and isokinetic knee strength (r = 0.640). Sex and BMI did not explain any significant variation in SWV. CONCLUSIONS: Ageing was associated with a decline in skeletal muscle stiffness which positively correlates with muscle weakness. Further research is needed to evaluate the promising role of SWE as a biomarker for sarcopenia assessment and potential falls risk prediction in elderly individuals.

Muscle shear wave elastography in idiopathic inflammatory myopathies: a case-control study with MRI correlation.

OBJECTIVE: To investigate muscle stiffness in patients with idiopathic inflammatory myopathies (IIM) using shear wave elastography (SWE) and to correlate the results with muscle strength and MRI features of myositis. MATERIALS AND METHODS: Muscle shear wave velocity (SWV) was measured in 23 active IIM patients (13 females, mean age 50.4 ± 16.1 years) and 23 matched healthy controls (13 females, mean age 50.7 ± 16.2 years). The investigated muscles included the vastus lateralis (VL), rectus femoris (RF), vastus medialis (VM) vastus intermedius (VI), biceps femoris (BF), semitendinosus (ST), semimembranosus (SM) and the biceps brachii (BB) scanned during relaxed resting and passive stretching positions. Participants performed multiple tests to evaluate their muscle strength. IIM patients had a thigh MRI to assess degrees of oedema, fatty infiltration and atrophy. RESULTS: In the resting position, IIM patients had a 12.9-22.2% significantly lower SWV (p < 0.05) for the quadriceps and hamstrings, but not BB. There was no difference during passive stretching. The SWV for VL, VI and BF showed moderate correlations with the muscle strength tests ranging from r = 0.47 to r = 0.70 (all p < 0.05). Lower SWV was associated with greater MRI scores of oedema (p = 0.001) and atrophy (p = 0.006). However, SWV did not correlate with fatty infiltration (r < 0.3; p = 0.28), creatine kinase (r = 0.28; p = 0.19) or disease duration (r = 0.26; p = 0.24). CONCLUSION: Shear wave elastography may detect abnormal reduced thigh stiffness in IIM patients. SWE measurements were significantly associated with muscle weakness and MRI signs of oedema and atrophy. Future research should investigate this new technology for monitoring disease activity.

Clinical presentation and therapeutic management of venous thrombosis in young children: a retrospective analysis.

BACKGROUND: Venous thromboembolism (VTE) in young children is not well documented. METHODS: Clinicians from 12 institutions retrospectively evaluated the presentation, therapeutic management, and outcome of VTE in children younger than 2 years seen in 2011-2016. Feasibility of recruiting these children in EINSTEIN-Jr. phase III, a randomized trial evaluating rivaroxaban versus standard anticoagulation for VTE, was assessed. RESULTS: We identified 346 children with VTE, of whom 227 (65.6%) had central venous catheter-related thrombosis (CVC-VTE), 119 (34.4%) had non-CVC-VTE, and 156 (45.1%) were younger than 1 month. Of the 309 children who received anticoagulant therapy, 86 (27.8%) had a short duration of therapy (i.e. < 6 weeks for CVC-VTE and < 3 months for non-CVC-VTE) and 17 (5.5%) had recurrent VTE during anticoagulation (n = 8, 2.6%) or shortly after its discontinuation (n = 9, 2.9%). A total of 37 (10.7%) children did not receive anticoagulant therapy and 4 (10.5%) had recurrent VTE.The average number of children aged < 0.5 years and 0.5-2 years who would have been considered for enrolment in EINSTEIN-Jr is approximately 1.0 and 0.9 per year per site, respectively. CONCLUSIONS: Young children with VTE most commonly have CVC-VTE and approximately one-tenth and one-fourth received no or only short durations of anticoagulant therapy, respectively. Recurrent VTE rates without anticoagulation, during anticoagulation or shortly after its discontinuation seem comparable to those observed in adults. Short and flexible treatment durations could potentially increase recruitment in EINSTEIN-Jr. phase III.

The effect of unit, depth, and probe load on the reliability of muscle shear wave elastography: Variables affecting reliability of SWE.

PURPOSE: There is currently no standardized method for muscle shear wave elastography (SWE). The objective of this study was to investigate the effect of unit of measurement, depth, and probe load on the reliability of muscle SWE. METHODS: The vastus lateralis, biceps femoris, biceps brachii, and abductor digiti minimi muscles were scanned on 20 healthy participants. The SWE readings were measured in shear wave velocity (m/s) and Young's modulus (kPa). Three acquisitions of varying depths were acquired from vastus lateralis. Minimal probe load was compared with the use of a standoff gel layer. Three repeated measurements were acquired to assess reliability using intraclass correlations (ICC). RESULTS: The mean elasticity varied across muscle groups and ranged from 1.54 m/s for biceps femoris to 2.55 m/s for abductor digiti minimi (difference = 1.01 m/s [95% confidence interval, CI = 0.92, 1.10]). Reporting readings in meters per second resulted in higher ICC of 0.83 (0.65, 0.93) in comparison to 0.77 (0.52, 0.90) for kilopascal for the vastus lateralis muscle only. Variance increased proportionally with depth reaching 0.17 (equivalent to ±0.82 m/s) at 6 cm. Using a standoff gel decreased ICC to 0.63 (0.20, 0.84) despite similar mean elasticity readings to minimal probe load. CONCLUSIONS: Different acquisition and technical factors may significantly affect the reliability of SWE in skeletal muscles. Readings acquired in the unit of shear wave velocity (m/s) from depths less than 4 cm using a minimal probe load without a standoff gel yielded the best reliability.

Mutations in TRNT1 cause congenital sideroblastic anemia with immunodeficiency, fevers, and developmental delay (SIFD).

Mutations in genes encoding proteins that are involved in mitochondrial heme synthesis, iron-sulfur cluster biogenesis, and mitochondrial protein synthesis have previously been implicated in the pathogenesis of the congenital sideroblastic anemias (CSAs). We recently described a syndromic form of CSA associated with B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD). Here we demonstrate that SIFD is caused by biallelic mutations in TRNT1, the gene encoding the CCA-adding enzyme essential for maturation of both nuclear and mitochondrial transfer RNAs. Using budding yeast lacking the TRNT1 homolog, CCA1, we confirm that the patient-associated TRNT1 mutations result in partial loss of function of TRNT1 and lead to metabolic defects in both the mitochondria and cytosol, which can account for the phenotypic pleiotropy.

Recommendations of the ESSR Arthritis Subcommittee on Ultrasonography in Inflammatory Joint Disease.

This article presents the recommendations of the European Society of Musculoskeletal Radiology Arthritis Subcommittee on the use of ultrasonography (US) in rheumatic disease, focused on the examination of joints in the adult population. The recommended examination technique and protocols used in a radiologic work-up are discussed. The main US features that can lead to a final diagnosis in the most common rheumatic diseases are addressed. The differential diagnosis that should be considered at image interpretation is presented. The role of US in interventional procedures and clinically important recent developments is also discussed.

Professional golfers' hips: prevalence and predictors of hip pain with clinical and MR examinations.

AIMS: This study aimed to determine the prevalence of hip pain in professional golfers, comparing the lead (left hip in right-handed golfer) and trail hips, and to establish what player characteristics predicted hip symptoms. METHODS: Male elite professional golf players were invited to complete questionnaires and undergo clinical and MR examinations while attending the Scottish Hydro Challenge 2015. Questionnaires determined player demographics, self-reported hip pain and an International Hip Outcome Tool 12 (iHOT12) score (hip-related quality of life). Clinical examinations determined hip range of motion and the presence of a positive impingement test. MR scans determined the presence of labral pathology and player hip morphology with measures of α angle (cam), acetabular depth (pincer) and femoral neck antetorsion. RESULTS: A total of 109 (70% of tournament field) of players completed questionnaires, 73 (47%) underwent clinical examination and 55 (35%) underwent MR examination. 19.3% of players reported of hip pain. 11.9% of lead and 9.1% of trail hips were painful (p=0.378), iHOT12 scores were lower in the lead (94.1) compared to the trail hip (95.3) (p=0.007). Stepwise multiple linear regression modelling was able to predict 20.7% of the variance in iHOT12 scores with mean α angles between 12 and 3 o'clock, and increasing age-significant variables (R(2)=0.207, p<0.001; ß=-0.502, p<0.001 and ß=-0.399, p=0.031, respectively). CONCLUSIONS: 19.3% of male professional golfers reported hip pain. The presence of an increasing α angle and increasing age were significant predictors of reduced hip-related quality of life.

Hip morphology in elite golfers: asymmetry between lead and trail hips.

AIM: During a golf swing, the lead hip (left hip in a right-handed player) rotates rapidly from external to internal rotation, while the opposite occurs in the trail hip. This study assessed the morphology and pathology of golfers' hips comparing lead and trail hips. METHODS: A cohort of elite golfers were invited to undergo MRI of their hips. Hip morphology was evaluated by measuring acetabular depth (pincer shape=negative measure), femoral neck antetorsion (retrotorsion=negative measure) and α angles (cam morphology defined as α angle >55° anteriorly) around the axis of the femoral neck. Consultant musculoskeletal radiologists determined the presence of intra-articular pathology. RESULTS: 55 players (mean age 28 years, 52 left hip lead) underwent MRI. No player had pincer morphology, 2 (3.6%) had femoral retrotorsion and 9 (16%) had cam morphology. 7 trail hips and 2 lead hips had cam morphology (p=0.026). Lead hip femoral neck antetorsion was 16.7° compared with 13.0° in the trail hip (p<0.001). The α angles around the femoral neck were significantly lower in the lead compared with trail hips (p<0.001), with the greatest difference noted in the anterosuperior portion of the head neck junction; 53° vs 58° (p<0.001) and 43° vs 47° (p<0.001). 37% of trail and 16% of lead hips (p=0.038) had labral tears. CONCLUSIONS: Golfers' lead and trail hips have different morphology. This is the first time side-to-side asymmetry of cam prevalence has been reported. The trail hip exhibited a higher prevalence of labral tears.

A novel syndrome of congenital sideroblastic anemia, B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD).

Congenital sideroblastic anemias (CSAs) are a heterogeneous group of inherited disorders identified by pathological erythroid precursors with perinuclear mitochondrial iron deposition in bone marrow. An international collaborative group of physicians and laboratory scientists collated clinical information on cases of CSA lacking known causative mutations, identifying a clinical subgroup of CSA associated with B immunodeficiency, periodic fevers, and development delay. Twelve cases from 10 families were identified. Median age at presentation was 2 months. Anemia at diagnosis was sideroblastic, typically severe (median hemoglobin, 7.1 g/dL) and markedly microcytic (median mean corpuscular volume, 62.0 fL). Clinical course involved recurrent febrile illness and gastrointestinal disturbance, lacking an infective cause. Investigation revealed B-cell lymphopenia (CD19⁺ range, 0.016-0.22 × 109/L) and panhypogammaglobulinemia in most cases. Children displayed developmental delay alongside variable neurodegeneration, seizures, cerebellar abnormalities, sensorineural deafness, and other multisystem features. Most required regular blood transfusion, iron chelation, and intravenous immunoglobulin replacement. Median survival was 48 months, with 7 deaths caused by cardiac or multiorgan failure. One child underwent bone marrow transplantation aged 9 months, with apparent cure of the hematologic and immunologic manifestations. We describe and define a novel CSA and B-cell immunodeficiency syndrome with additional features resembling a mitochondrial cytopathy. The molecular etiology is under investigation.

Do MRI and ultrasound of the anterior pelvis correlate with, or predict, young football players' clinical findings? A 4-year prospective study of elite academy soccer players.

AIMS: To prospectively follow a cohort of elite young male professional soccer players with sequential symptom questionnaires and imaging of the anterior pelvis to determine the prevalence and severity of imaging findings. METHODS: 34 male athletes (mean age 16.5 years) underwent clinical examination, history/symptom questionnaire, ultrasound and 1.5 T MRI of the anterior pelvis. Athletes then underwent annual questionnaire and ultrasound with MRI also performed every 18 months. Two experienced radiologists scored ultrasound (consensus) and MRI (independently) for abnormality including pubic bone, capsule and tendon oedema and scores correlated with symptoms and presence or absence of previous injuries. RESULTS: Over 4 years the participants fell from 34 to 22 in number with no withdrawals due to groin injury. On study entry no athletes had undergone previous hip or pelvic surgery. On MRI pubic bone oedema, secondary cleft, capsule/tendon oedema and enhancement did not differ substantively between players with and without history of previous injury. κ Analysis for MRI scoring showed excellent agreement (0.84-0.96) for pubic bone marrow oedema, secondary cleft, capsule/tendon oedema and enhancement. On ultrasound inguinal wall motion and adductor tendinopathy did not differ substantively between players with and without history of previous injury. Stability of imaging assessments over time showed no consistent difference. CONCLUSIONS: Pubic bone marrow and parasymphyseal findings (cleft, capsule/tendon oedema) on MRI or inguinal canal ballooning on ultrasound were frequently found in asymptomatic athletes and did not predict injury or symptom development.

EULAR recommendations for the use of imaging of the joints in the clinical management of rheumatoid arthritis.

OBJECTIVE: To develop evidence-based recommendations on the use of imaging of the joints in the clinical management of rheumatoid arthritis (RA). METHODS: The task force comprised an expert group of rheumatologists, radiologists, methodologists and experienced rheumatology practitioners from 13 countries. Thirteen key questions on the role of imaging in RA were generated using a process of discussion and consensus. Imaging modalities included were conventional radiography, ultrasound, MRI, CT, dual-emission x-ray absorptiometry, digital x-ray radiogrammetry, scintigraphy and positron emission tomography. Research evidence was searched systematically for each question using MEDLINE, EMBASE and Cochrane CENTRAL. The experts used the evidence obtained from the relevant studies to develop a set of 10 recommendations. The strength of recommendation was assessed using a visual analogue scale. RESULTS: A total of 6888 references was identified from the search process, from which 199 studies were included in the systematic review. Ten recommendations were produced encompassing the role of imaging in making a diagnosis of RA, detecting inflammation and damage, predicting outcome and response to treatment, monitoring disease activity, progression and remission. The strength of recommendation for each proposition varied according to both the research evidence and expert opinion. CONCLUSIONS: Ten key recommendations for the role of imaging in the management of RA were developed using research-based evidence and expert opinion.

Congenital amegakaryocytic thrombocytopenia (CAMT) presenting as severe pancytopenia in the first month of life.

Congenital amegakaryocytic thrombocytopenia (CAMT) is characterised by neonatal thrombocytopenia, with reduced or absent bone marrow megakaryocytes, leading eventually to pancytopenia. The mean age for progression to bone marrow failure is four years, with the earliest reported being six months. We describe a CAMT patient with compound heterozygous mutations of the causative MPL gene (one being a previously unreported splice site mutation in intron 11) who developed pancytopenia within the first month of life. This report emphasises the importance of considering CAMT in the differential diagnosis of congenital aplastic anaemia or idiopathic aplastic anaemia in babies.