SP0140 Imaging Techniques in Pediatric Connective Tissue Disease – Which and When?
Authors: P. Collado
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Systemic lupus erythematosus</h3> Renal disease and haematological involvement are common in Juvenile SLE, but musculoskeletal symptoms are often present and can cause substantial morbidity. Several studies have demonstrated that ultrasonography might be convenient in visualizing the involvement of peri- and intraarticular soft tissues in JSLE as well as in adults. Osteonecrosis occurs in 10–20% of JSLE. Plain films and MRI should be considered for the diagnosis of avascular necrosis (AVN) in a JSLE patient with painful hip/limp who had been treated with steroids for long periods. Nailfold capillaroscopy (NFC) is a useful technique to differentiate primary from secondary Raynaud9s phenomenon associated to several connective tissue diseases, particularly in girls with JSLE. <h3>Idiopathic inflammatory myopathy in childhood</h3> JDM is the commonest inflammatory myopathy of childhood. Diagnostic criteria continue to be based on those of Bohan and Peter, but an international survey in 2006 proposed adding items such as MRI and NFC. Historically, MRI techniques used to evaluate muscle disorders in children have included T1-weighted images (T1WI) and water-sensitive sequences (i.e., short-tau inversion recovery (STIR) or T2-weighted images (T2WI), with or without fat suppression). These latter sequences are very sensitive to the presence of intramuscular oedema reflecting inflammation, whereas fatty infiltration and muscle atrophy can be detected on T1WI in chronically affected muscles. Whole-body MRI is useful in the assessment of disease activity in JDM as well as guiding muscle biopsy. Recent MR techniques have expanded the use of MR imaging to include quantitative analysis. Ultrasound may be a cost-effective alternative to MRI, with contrast-enhanced ultrasound (CEUS) also permitting the assessment of muscle vascularization. With ultrasound elastography, the relative tissue stiffness can be visualised. In Raynaud9s phenomenon (RF), NFC can early demonstrate some typical capillary changes in patients with inflammatory myopathy that it has been called “scleroderma-like pattern”. <h3>Sclerodermas</h3> Scleroderma is a rare condition in children. There are 2 main distinctive categories: juvenile systemic sclerosis (SSc) and juvenile localized scleroderma. <i>Localized Scleroderma</i> is more frequent than SSc in children but still an uncommon condition. US can demonstrate changes in dermal thickness and level of echogenicity that seems to correlate with disease activity. NFC shows typical capillary changes in RF. Unlike adults, in juvenile-onset SSc the overall capillaroscopic pattern is called “scleroderma pattern” and the classification in “early”, “active” and “late” pattern has not common been applied. During the last years, various new technologies (infrared thermography [IT], laser doppler flowmetry [LDF], high-frequency Gray Scale-ultrasound, elastography, MRI and high-resolution computed tomography thorax) have been developed for better identifying and managing of disease. Because active disease can persist for years and can involved systemic structures, recommendations for screening for cardiopulmonary involvement in JSS have recently been discussing by PRES Scleroderma Working Group. <h3>S. Sjögren and mixed connective tissue disease</h3> Primary Sjögren9s syndrome (pSjS) is a systemic autoimmune disease uncommon in children. Secondary SjS has a more frequent occurrence in childhood, most commonly accompanies SLE, or less often accompanies another Connective Tissue Disease (CTD). Since salivary gland (GS) biopsy is considered as an invasive technique for children, ultrasonography could be a kind tool to provide information about parotid and major salivary glands morphology. In US assessment of SjS, parotid echogenecity is compared to thyroid echogenecity. Some studies on adult pSjS have described a typical pattern on SGUS characterised by lost of the homogeneous internal echogenicity and echotexture, with multiple oval hypoechoic areas of different size. Similar SGUS changes have been reported in juvenile pSjS. <h3>References</h3> Demirkaya E. Musculoskeletal sonography in juvenile systemic lupus erythematosus Arthritis Care Res 2009;61:58–60 Malattia C. Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis. Ann Rheum Dis 2014;73:1083–1090 Ingegnoli F. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: a EUSTAR exploratory study. Microvascular Research 2015: 102:19–24 Li S. Sonographic evaluation of pediatric localized scleroderma: preliminary disease assessment measures Pediatric Rheumatology 2010, 8:14 Nieto JC. Salivary gland ultrasound in children: a useful tool in the diagnosis of juvenile Sjögren9s síndrome. Clin Exp Rheum 2014;32 <h3>Disclosure of Interest</h3> None declared