Maria Beatrice Damasio

Synovial and inflammatory diseases in childhood: role of new imaging modalities in the assessment of patients with juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) represents a group of heterogeneous diseases characterized by a chronic inflammatory process primarily targeting the synovial membrane. A persistent synovitis is associated with an increased risk of osteocartilaginous damage.With the advent of effective structure-modifying treatment for JIA, it may be possible to significantly reduce or even completely prevent structural damage and associated functional disability. The trend towards early suppression of inflammation, in order to prevent erosive disease, shifts the emphasis away from conventional radiographic detectable structural damage to the slightest traces of early joint damage, and drives the need for alternative imaging techniques more sensitive in detecting early signs of disease activity and damage. In this regard MRI and US are playing an increasing role in the evaluation of arthritic joints.This article will review the key aspects of the current status and recent important advances of imaging techniques available to investigate the child with rheumatic disease, briefly discussing conventional radiography, and particularly focusing on MRI and US. In this era of advancing imaging technology, knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with JIA.

Dynamic contrast-enhanced magnetic resonance imaging in the assessment of disease activity in patients with juvenile idiopathic arthritis

OBJECTIVE To determine the capability and reliability of dynamic contrast-enhanced MRI (DCE-MRI) in the assessment of disease activity in juvenile idiopathic arthritis (JIA). METHODS DCE-MRI of the clinically more affected wrist or hip joints was undertaken in 21 patients, coupled with standard clinical assessment and biochemical analysis. Synovial inflammation was assessed by computing the maximum level of synovial enhancement (ME), the maximum rate of enhancement (MV) and the rate of early enhancement (REE) from the enhancement curves generated from region of interest independently delineated by two readers in the area of the ME. Correlations between dynamic parameters and clinical measures of disease activity, and static MRI synovitis score were investigated. RESULTS In patients with wrist arthritis, REE correlated with the wrist swelling score (r(s) = 0.72), ESR (r(s) = 0.69), pain assessment scale (r(s) = 0.63) and childhood HAQ (r(s) = 0.60). In patients with hip arthritis, ME correlated with the hip limitation of motion (r(s) = 0.69). Static MRI synovitis score based on post-gadolinium enhancement correlated with MV (r(s) = 0.63) in patients with wrist arthritis and with ME (r = 0.68) in those with hip arthritis. The inter-reader agreement assessed by intra-class correlation coefficient (ICC) for ME, MV and REE (ICC = 0.98, 0.97 and 0.84, respectively) was excellent. CONCLUSIONS DCE-MRI represents a promising method for the assessment of disease activity in JIA, especially in patients with wrist arthritis. As far as we know, this study is the first to demonstrate the feasibility, reliability and construct validity of DCE-MRI in JIA. These results should be confirmed in large-scale longitudinal studies in view of its further application in therapeutic decision making and in clinical trials.

Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis

Objective To compare whole-body MRI (WB-MRI) with clinical examination in the assessment of disease activity in juvenile dermatomyositis (JDM). Methods WB-MR images were obtained from 41 JDM patients and 41 controls using a 1.5 T MRI scanner and short τ inversion recovery sequences. 18 patients had follow-up WB-MRI. Muscle, subcutaneous tissue and myofascial signal abnormalities were scored in 36 muscular groups and on proximal and distal extremities. WB-MRI and clinical assessments were performed concurrently and results compared. Validation procedures included analysis of feasibility, reliability, construct validity, discriminative ability and responsiveness. Results WB-MRI revealed distal legs (26/41 patients) and forearm (19/41 patients) muscle inflammation undetected during clinical examination and allowed an accurate assessment of subcutaneous (23/41 patients) and myofascial involvement (13/41 patients). 27 patients showed a patchy distribution of muscle inflammation while in seven the abnormal hyperintense areas tended to be homogeneously distributed. The inter-reader agreement for muscular, subcutaneous and myofascial WB-MRI scores was excellent. Correlations between WB-MRI muscle score and disease activity measures were excellent (Manual Muscle Test: rs=−0.84, Childhood Myositis Assessment Scale: rs=−0.81). WB-MRI score was higher in JDM active patients when compared with the control group (pB<0.0001) and the inactive patients (pB=0.004), and showed an excellent responsiveness (standardised response mean=1.65). Follow-up WB-MRI showed resolution of inflammation in nine patients whereas clinical criteria for remission were satisfied in five. Conclusions WB-MRI provides additional information to clinical evaluation and represents a promising tool to estimate total inflammatory burden, tailor treatment and monitor its efficacy.

Development and preliminary validation of a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis

Objectives To develop and validate a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis (JIA). To compare the paediatric MRI score with the adult-designed. Outcome Measures in Rheumatology Clinical Trials—Rheumatoid Arthritis MRI Score (RAMRIS), whose suitability for assessing growing joints was tested. Methods In 66 patients with JIA the clinically more affected wrist was studied. Thirty-nine patients had a 1-year MRI follow-up. Two readers independently assigned the paediatric score and the RAMRIS to all studies. Validation procedures included analysis of reliability, construct validity and responsiveness to change. A reduced version of the bone erosion score was also developed and tested. Results The paediatric score showed an excellent reproducibility (interclass correlation coefficient >0.9). The interobserver agreement of RAMRIS was moderate for bone erosions and excellent for bone marrow oedema (BMO). The paediatric score and RAMRIS provided similar results for construct validity. The responsiveness to change of the paediatric score was moderate for synovitis and bone erosion, and poor for BMO and did not improve when RAMRIS was applied. The reduced version of the bone erosion was valuable for the assessment of joint damage, and provided time-saving advantages. Conclusion The results demonstrate that the paediatric MRI score is a reliable and valid method for assessing disease activity and damage in JIA. Unexpectedly, the RAMRIS provides acceptable suitability for use in the paediatric age group. Further work, especially in a longitudinal setting, is required before defining the most suitable MRI scale for assessing growing joints.

ESPR uroradiology task force and ESUR paediatric working group: imaging and procedural recommendations in paediatric uroradiology, part III. Minutes of the ESPR uroradiology task force minisymposium on intravenous urography, uro-CT and MR-urography in childhood

The ESPR working groups have addressed intravenous urography (IVU), uro-CT and MR-urography (MRU) in childhood as the last in the series of recommendations for paediatric uroradiology. The aim of this process was again to standardise paediatric uroradiologic imaging and to reduce invasiveness and radiation dose. As for the existing recommendations, the new proposals are consensus-based because evidence is lacking, use and indications have changed, or approaches on how to perform the examination in children differ in the literature (MRU). As in the previous recommendations, a thorough review of the literature and existing guidelines and recommendations has been performed. The proposals were discussed within the group and with non-member experts and colleagues from other partner disciplines.These recommendations aim to serve as a quality measure in order to standardise the procedures and thus grant comparable good quality results throughout different institutions.

ESPR Uroradiology Task Force and ESUR Paediatric Working Group—Imaging recommendations in paediatric uroradiology, Part V: childhood cystic kidney disease, childhood renal transplantation and contrast-enhanced ultrasonography in children

The ESPR Uroradiology Task Force and the ESUR Paediatric Working Group present two new recommendations on imaging in childhood cystic kidney disease and in childhood renal transplantation, and address the presently restricted availability of contrast-enhanced (ce) US in children. New insights into the genetics require an updated classification of paediatric cystic kidney disease along with a new concept of diagnostic imaging. Characteristic imaging features are key to the new classification. Available recommendations for imaging renal transplantation in children are not satisfactory. The following consensus-based algorithm proposes a more effective and more uniform imaging concept, reducing invasiveness, enhancing diagnostic accuracy, and facilitating future multicentre studies and meta-analysis. At present, ce-US in children can only be performed off-license, since the only approved US contrast agent (CA) for children has been taken off the market. Nevertheless, paediatric ce-US is practiced at multiple places using Sonovue® (Bracco, Milan, Italy), a generally available agent in Europe. From a medical and scientific perspective, paediatric ce-US should be promoted, and efforts are undertaken to collect data on paediatric US-CA applications. Routine paediatric imaging depends on local expertise and availability of equipment. The imaging recommendations and supportive data are intended to ease the physicians’ difficult task of dealing with the specific diagnostic demands of paediatric paediatric cystic kidney disease and transplantation.

Paediatric musculoskeletal US beyond the hip joint

US is a technique particularly suited to the investigation of musculoskeletal disorders in children and adolescents. This review paper describes a range of clinical settings beyond the hip joint where US has a significant role to play, including sports injuries, infectious diseases, inflammatory and degenerative conditions, congenital and developmental disorders, acute trauma of bone and joints, and peripheral nerve injuries. In some circumstances, US can be regarded as the most effective means of diagnostic imaging, whereas in other instances, it is an alternative or supplement to other more comprehensive imaging modalities, like MRI and CT. Although MRI offers superior soft-tissue contrast resolution, US is low-cost, non-invasive and has higher spatial resolution and real-time capability for the assessment of musculoskeletal structures during joint movement and stress manoeuvres.

MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study

BackgroundMRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA).ObjectiveThe purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study.Materials and methodsWrist MRI was performed in 76 children with JIA. On postcontrast 3-D spoiled gradient-echo and fat-suppressed T2-weighted spin-echo images, joint recesses were scored for the degree of synovial enhancement, effusion and overall inflammation independently by two paediatric radiologists. Total-enhancement and inflammation-synovitis scores were calculated.ResultsInterobserver agreement was poor to moderate for enhancement and inflammation in all recesses, except in the radioulnar and radiocarpal joints. Intraobserver agreement was good to excellent. For enhancement and inflammation scores, mean differences (95 % CI) between observers were −1.18 (−4.79 to 2.42) and −2.11 (−6.06 to 1.83). Intraobserver variability (reader 1) was 0 (−1.65 to 1.65) and 0.02 (−1.39 to 1.44).ConclusionIntraobserver agreement was good. Except for the radioulnar and radiocarpal joints, interobserver agreement was not acceptable. Therefore, the proposed scoring system requires further refinement.

MRI assessment of bone marrow in children with juvenile idiopathic arthritis: intra- and inter-observer variability

BackgroundBone marrow oedema (BMO) is included in MRI-based scoring systems of disease activity in adults with rheumatoid arthritis. Similar systems in juvenile idiopathic arthritis (JIA) are lacking.ObjectiveTo assess the reproducibility in a multi-centre setting of an MRI BMO scoring system in children with JIA.Materials and methodsSeventy-six wrist MRIs were read twice, independently, by two experienced paediatric radiologists. BMO was defined as ill-defined lesions within the trabecular bone, returning high and low signal on T2- and T1-weighted images respectively, with or without contrast enhancement. BMO extension was scored for each of 14 bones at the wrist from 0 (none) to 3 (extensive).ResultsThe intra-observer agreement was moderate to excellent, with weighted kappa ranging from 0.85 to 1.0 and 0.49 to 1.0 (readers 1 and 2 respectively), while the inter-observer agreement ranged from 0.41 to 0.79. The intra- and inter-observer intraclass correlation coefficients were excellent and satisfactory, respectively.ConclusionThe scoring system was reliable and may be used for grading bone marrow abnormality in JIA. The relatively large variability in aggregate scores, particularly between readers, underscores the need for thorough standardisation.

Novel automated system for magnetic resonance imaging quantification of the inflamed synovial membrane volume in patients with juvenile idiopathic arthritis

To introduce a novel automated method for the quantification of the inflamed synovial membrane volume (SV) using magnetic resonance imaging (MRI), and to investigate its feasibility and validity in patients with juvenile idiopathic arthritis (JIA).