- [Role of magnetic resonance imaging in the diagnosis of juvenile dermato-myositis and polymyositis in Chinese children]. [Journal Article]
- ZEZhonghua Er Ke Za Zhi 2016 Oct 2; 54(10):767-772
- Objective: To evaluate the utility of magnetic resonance imaging (MRI) in diagnosis of juvenile dermatomyositis and polymyositis (JDM-PM) in children. Method: Fifty-four patients with JDM-PM in the a...
Objective: To evaluate the utility of magnetic resonance imaging (MRI) in diagnosis of juvenile dermatomyositis and polymyositis (JDM-PM) in children. Method: Fifty-four patients with JDM-PM in the active stage were enrolled in the study group. Twelve patients with benign acute childhood myositis and forty patients with juvenile idiopathic arthritis (JIA) complicated with myositis were enrolled as controls. MRI imaging of thighs was performed in all patients, fast spin echo T1WI, T2WI, and STIR were obtained in all patients.Muscle biopsy was performed in 41/54 patients with JDM-PM. We compared the value of MRI in diagnosis of JDM-PM with muscle biopsy, electromyography and serum aspartate transaminase (AST), alanine transaminase (ALT), creatine kinase (CK), isoenzyme of creatine kinase (CKMB), lactate dehydrogenase (LDH), hydroxybutyrate dehydrogenase (HBDH) levels. Continuous normally distributed variables were reported as means and continuous non-normally distributed variables as median. Chi-square test and Fisher exact test were used to test differences between MRI and other categorical variables. Result: A total of 54 patients were included. Twenty-seven patients were male and the others were female. Average age of the patients was (7.1±3.5) years (2-13 years); 45(83%) paitests were JDM cases and 9(17%) patients had JPM. All patients had MRI examination. Of the 54 patients, 53 had multiple myositis; 10 out of 50 (19%) patients received second MRI after treatment, 6 out of 10 patients had normal findings, 4 patients showed obviously improved images; 41 out of 54 patients underwent muscle biopsy; 22 out of 41 patients had inflammatory cells infiltration and muscle fiber degeneration. The results of the muscle enzyme tests are as follows: 27 (50%) patients had elevated AST, 24 (44%) patients had elevated ALT, 22 (41%) patients had elevated CK, 18(33%) patients had elevated CKMB, and LDH rose in 30 (56%) patients, HBDH rose in 28(52%) patients. These results suggested that muscle MRI was more sensitive than muscle biopsy and muscle enzyme tests in diagnosis of JDM-PM. Conclusion: Patients with JDM-PM showed diffuse patchy hyperintense signals on T2WI of their thighs. MRI may be a sensitive, reliable, and noninvasive tool for clinical diagnosis and theraputic evaluation of JDM-PM.
- Information technology in paediatric rheumatology. [Review]
- CEClin Exp Rheumatol 2016 Sep-Oct; 34 Suppl 101(5):11-16
- Information technology in paediatric rheumatology has seen several exciting developments in recent years. The new multidimensional questionnaires for juvenile idiopathic arthritis, juvenile dermatomy...
Information technology in paediatric rheumatology has seen several exciting developments in recent years. The new multidimensional questionnaires for juvenile idiopathic arthritis, juvenile dermatomyositis, and juvenile autoinflammatory diseases integrate all major parent- and child-reported outcomes (PCROs) used in these diseases into a single tool, and provide an effective guide to manage, document change in health, assess effectiveness of therapeutic interventions, and verify the parent and child satisfaction with illness outcome. The Pharmachild registry is aimed to gain information concerning the long-term effectiveness and safety of the medications currently used in juvenile idiopathic arthritis, particularly biologic agents, through collection of prospective data in a large, multinational sample of patients. Children and their parents are directly involved in the data collection by means of the regular completion of a digital version of a multidimensional questionnaire. The Patient-Reported Outcomes Measurement Information System (PROMIS) employs modern measurement science to advance assessment of PCROs, particularly HRQL, and offers multidimensional profile measures. The conceptual link of paediatric PROMIS with adult instruments facilitates harmonisation of assessments made in children and adolescents with those carried out in young adults in the process of transition of medical care. Development of electronic versions of questionnaires that permit their completion through smartphones or touch-screen devices will revolutionise information collection from parents and children, foster the regular collection of PCROs in routine care, and ultimately improve the quality of self-reported health data, and patient outcomes.
- Myositis-specific autoantibodies and their association with malignancy in Italian patients with polymyositis and dermatomyositis. [Journal Article]
- CRClin Rheumatol 2016 Oct 20
- This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 pa...
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis. In six dermatomyositis cases, we found five solid forms of cancer and one Hodgkin's lymphoma in long-term remission. Among patients with cancer-associated dermatomyositis, three were positive for anti-TIF1γ/α, two for anti-Mi-2, and one for anti-MJ/NXP-2. The strongest positivity of anti-TIF1γ was seen in two active forms of cancer, and this antibody was either negative or positive at low titers in the absence of cancer or in the 7-year remission Hodgkin's lymphoma. Four out of twenty (20 %) patients with polymyositis had solid cancer, but no specific association with autoantibodies was identified; further, none of the four cases of antisynthetase syndrome had a history of cancer. No serum myositis-associated autoantibody was observed in control sera, resulting in positive predictive value 75 %, negative predictive value 78.5 %, sensitivity 50 %, specificity 92 %, and area under the ROC curve 0.7083 for the risk of paraneoplastic DM in anti-TIF1γ/α (+) patients. Myositis-specific autoantibodies can be identified thanks to the use of immunoprecipitation, and their association with cancer is particularly clear for anti-TIF1γ/α in dermatomyositis. This association should be evaluated in a prospective study by immunoprecipitation in clinical practice.
- Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies. [Review]
- RORMD Open 2016; 2(2):e000291
- Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are character...
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention. The risk of developing atherosclerotic coronary artery disease is increased twofold to fourfold in DM/PM. New and improved diagnostic methods have in recent studies in PM/DM and JDM demonstrated a high prevalence of subclinical cardiac involvement, especially diastolic dysfunction. Interactions between proinflammatory cytokines and traditional risk factors might contribute to the pathogenesis of cardiac dysfunction. Heart involvement could also be related to myocarditis and/or myocardial fibrosis, leading to arrhythmias and congestive heart failure, demonstrated both in adult and juvenile IIM. Also, reduced heart rate variability (a known risk factor for cardiac morbidity and mortality) has been shown in long-standing JDM. Until more information is available, patients with IIM should follow the same recommendations for cardiovascular risk stratification and prevention as for the corresponding general population, but be aware that statins might worsen muscle symptoms mimicking myositis relapse. On the basis of recent studies, we recommend a low threshold for cardiac workup and follow-up in patients with IIM.
- Current Perspectives on Imaging for Systemic Lupus Erythematosus, Systemic Sclerosis, and Dermatomyositis/Polymyositis. [Review]
- RDRheum Dis Clin North Am 2016; 42(4):711-732
- Systemic rheumatic diseases frequently pose diagnostic challenges to the clinician. The precise nature of the disorder can be obscure and different disorders can present with similar symptoms, such a...
Systemic rheumatic diseases frequently pose diagnostic challenges to the clinician. The precise nature of the disorder can be obscure and different disorders can present with similar symptoms, such as joint pain. Plain radiographs provide an appropriate starting point for detection of joint abnormalities. Musculoskeletal ultrasound and MRI offer greater sensitivity in detecting synovitis, tenosynovitis and bony erosions, among other features. However, due to the rarity of these diseases and lack of prospective longitudinal trials, a broader picture of the epidemiology of these findings and their implications for treatment and outcomes remains to be determined.
- Tocilizumab in the treatment of mixed connective tissue disease and overlap syndrome in children. [Journal Article]
- RORMD Open 2016; 2(2):e000271
- Arthritis is one of the main manifestations of mixed connective tissue disease (MCTD) and overlap syndrome in children and can be responsible for functional disability. We report on 2 children with a...
Arthritis is one of the main manifestations of mixed connective tissue disease (MCTD) and overlap syndrome in children and can be responsible for functional disability. We report on 2 children with arthritis that were dramatically improved by a treatment with interleukin-6 (IL-6) blockers in the context of connective tissue disease. However, in both cases, other systemic autoimmune symptoms were not modified by the treatment and autoantibodies tend to increase, suggesting a differential effect of IL-6 inhibition on articular inflammation and systemic autoimmunity.
- Imaging of Pulmonary Manifestations of Connective Tissue Diseases. [Review]
- RCRadiol Clin North Am 2016; 54(6):1015-1031
- Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rh...
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia. Drug treatment of CTDs can cause complications, including opportunistic infection.
- Whole-body MRI for full assessment and characterization of diffuse inflammatory myopathy. [Journal Article]
- ARActa Radiol Open 2016; 5(9):2058460116668216
- CONCLUSIONS: WB-MRI is the diagnostic modality of choice for cases of inflammatory myopathy. It accurately detects the most severely affected muscles candidate for biopsy and provides a reliable baseline study for follow-up of disease progression as well as response to treatment.
- Enzyme-linked immunosorbent assays for detection of anti-transcriptional intermediary factor-1 gamma and anti-Mi-2 autoantibodies in dermatomyositis. [Journal Article]
- JDJ Dermatol Sci 2016 Sep 23
- CONCLUSIONS: The current study demonstrates the utility of newly established ELISAs for anti-TIF1γ and anti-Mi-2β Abs, which can serve as easier detection systems for routine testing.
New Search Next
- Autoantibody to scaffold attachment factor B (SAFB): A novel connective tissue disease-related autoantibody associated with interstitial lung disease. [Journal Article]
- JAJ Autoimmun 2016 Sep 24
- CONCLUSIONS: Anti-SAFB Ab is a novel CTD-related autoAb possibly associated with ILD.