Background: Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children.

Objective: To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI.

Materials And Methods: Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images.

Results: Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31%, respectively, with a sensitivity of 40% and specificity of 67%.

Conclusion: Compression-strain US elastography does not accurately detect active myositis in children with juvenile idiopathic inflammatory myopathy and cannot replace MRI as the imaging standard for detecting myositis in these children. The association between abnormal US elastography and increased muscle echogenicity suggests that elastography is capable of detecting muscle derangement in patients with myositis; however further studies are required to determine the clinical significance of these findings.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00247-015-3350-8DOI Listing

Publication Analysis

Top Keywords

active myositis
16
muscle echogenicity
16
juvenile idiopathic
12
idiopathic inflammatory
12
inflammatory myopathy
12
active disease
12
elastography
11
elastography detecting
8
detecting active
8
myositis children
8

Similar Publications

Idiopathic inflammatory myopathies (IIM), or myositis, are a heterogeneous group of autoimmune disorders that can affect multiple organs, including the muscles, skin, joints, lungs, heart, and gastrointestinal tract. While new-onset myositis has been reported following SARS-CoV-2 infection, cases associated with COVID-19 vaccination remain rare. We describe a unique case of severe progressive edematous facial myositis resembling angioedema in a 22-year-old man, with onset one to two weeks after receiving dual SARS-CoV-2 and influenza vaccinations.

View Article and Find Full Text PDF

The combination of severe myalgia, progressive weakness, and blood in the urine often leads a neurologist to consider myositis. Accordingly, reddish urine may be linked to urine myoglobinuria brought about by muscle destruction. Nevertheless, in a young patient with normal creatine kinase complaining of immobility, adult-onset Still's disease (AOSD) should be one of the top differentials.

View Article and Find Full Text PDF

Introduction: To identify the most effective treatment for juvenile dermatomyositis (JDM), considering efficacy, safety, impact on patients and improvement in their quality of life.

Material And Methods: A systematic review was carried out comparing known treatments and immunobiological therapies, evaluating clinical improvement, adverse events and prognosis. The MEDLINE, PubMed, LILACS and Cochrane Library databases were used with children aged 0 to 18 diagnosed with JDM.

View Article and Find Full Text PDF

Systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and idiopathic inflammatory myositis (IIM) are autoimmune diseases managed with long-term immunosuppressive therapies. Hu19-CD828Z, a fully human anti-CD19 chimeric antigen receptor (CAR) with a CD28 costimulatory domain, is engineered to potently deplete B-cells. In this study, we manufactured Hu19-CD828Z CAR T-cells from peripheral blood of SLE, IIM, and SSc patients and healthy donors (HDs).

View Article and Find Full Text PDF

Background: Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myositis. Anti-signal recognition particle (SRP) antibodies are highly specific to this disease.

Case: A 76-year-old woman presented with a 4-month history of acute progressive limb muscle weakness and dysphagia.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!