Management Of Juvenile Dermatomyositis Research Articles

Early-onset juvenile dermatomyositis: A tertiary referral center experience and review of the literature

Background/ObjectivesThe aim of our study is twofold: To evaluate the presentation, diagnosis, clinical course, and management of juvenile dermatomyositis (JDM) in children under three years of age, and to compare with older-onset patients. MethodsNine patients with early-onset, and 63 patients with older-onset JDM followed between December 2010 and April 2022 are included. We also reviewed the literature on early-onset JDM from the inceptions of the PubMed/MEDLINE and Scopus databases up to April 1st, 2022. ResultsEarly-onset JDM patients were characterized by longer median diagnostic delay (p = 0.005), calcinosis (p = 0.006), anti-NXP2 antibody (p = 0.049). Diagnostic pathway included muscle biopsy (77.7% versus 50.8%). Muscle biopsy findings were more severe in the early-onset group (p<0.001). Although there was no difference in the partial and complete remission rates, the relapse rate was significantly higher in the early-onset group (p = 0.001), reflected to requirement of intravenous immunoglobulin (p = 0.001), cyclophosphamide (p = 0.011), and biological agents (p = 0.016). Literature search revealed 32 articles reporting 75 patients. The median diagnostic delay was 5 (1–30) months. Calcinosis was present in 29.5%. Twenty-three of the 44 patients (52.3%) had a muscle biopsy. Forty-one patients (64.1%) received second and third-line treatments. Complete remission was achieved in almost half of these patients (48.9%), but relapse was observed in 75%. The mortality rate was 10.2%. ConclusionDiagnosis can be challenging and delayed in early-onset JDM patients. Compared to older-onset JDM patients, this group had higher relapse rate, more severe muscle biopsy findings, and received intensive immunosuppressive treatment.

42 Egyptian evidence-based consensus recommendations for diagnosis and targeted management of juvenile dermatomyositis. An initiative by the Egyptian College of Pediatric Rheumatology

BackgroundJuvenile dermatomyositis (JDM) is the most common type of juvenile idiopathic inflammatory myopathy mainly affecting the skin and proximal muscles. To date, there are no Egyptian nationally agreed, evidence-based guidelines concerning the diagnosis and management of JDM in children and treatment is often based on clinical expertise. These facts prompted us to develop clinical practice guidance for JDM suitable for patients in Egypt. This work was an initiative by the Egyptian College of Pediatric Rheumatology aiming at optimizing the management approaches for children and young adults with JDM.ObjectiveTo provide evidence-based consensus recommendations for diagnosis and management of JDM.MethodsThis study was carried out to achieve an Egyptian expert consensus on a management strategy for JDM using Delphi technique. A multistep process strategy was adopted, which started by developing 16 key clinical questions by scientific committee according to the Patient/Population, Intervention, Comparison, Outcomes and Time (PICOT) approach. The core leadership team identified clinicians and researchers with expertise in pediatric rheumatology all over Egypt. An evidence-based, systematic, literature review was conducted to compile evidence for the JDM management. Delphi process was implemented (3-rounds) to reach a consensus.ResultsTwenty-five expert panel participated in the 3 rounds with response rate 100%. A total of 22 recommendations, categorized into 15 domains (Targeted population, diagnostic criteria, high risk patients, diagnostic criteria, definition of disease activity status, differences between JDM and DM in adults, lab tests, treatment targets, treatment, monitoring, resisted cases, drug tapering, comorbidities, prognosis, vaccination and transition program. The Agreement with the recommendations (rank 7–9) ranged from 85.2–95.2%. The Consensus was reached (i.e. ≥75% of respondents strongly agreed or agreed) on all the clinical standards. Algorithm for management has also been developed.ConclusionThis work provided an updated management approach for JDM patients. This evidence-based informed consensus process is expected to support uniform, high quality standards of care for children with JDM in EgyptThe implication to policy, practice, research and advocacyto provide updated recommendations for better management of JDM.

Multispecialty approach for improving outcomes in juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a pediatric rheumatic disease characterized by inflammation of the muscle and skin. Prognosis of JDM in children has improved in general owing to medical progress; however, pathogenesis and management of JDM in children and prognosis in refractory JDM remain challenging. For elucidation of JDM pathophysiology and establishment of appropriate treatment for JDM, pediatric rheumatologists need to adopt a multispecialty approach that involves experts in genetics, immunology, pathology, musculoskeletal medicine, dermatology, pulmonology, cardiology, hematology, gastroenterology, endocrinology, ophthalmology, psychology, radiology, pharmacology, physiotherapy, surgery, preventive medicine, and adult rheumatology. Such collaborations will potentially lead to improved outcomes in children with JDM.

Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists

BackgroundJuvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course. Data on the optimal management are mostly lacking. Currently treatment decisions are often based on experts’ opinions. In order to develop consensus-based treatment strategies for JDM in Germany a survey was pursued to analyze the current clinical practice.MethodsAn online survey addressing all members of the Society for Pediatric Rheumatology (GKJR) in Germany and Austria and pediatric neurologists with expertise in JDM was performed in February/March of 2016. The questionnaire consisted of 5 case scenarios including diagnostic criteria, treatment of moderate, severe and refractory JDM, using either multiple choice or a 5-point Likert scale. Basic descriptive statistics were used to analyze the findings.ResultsThe survey was completed by 60 pediatric rheumatologists and 7 pediatric neurologists experienced in the management of JDM. Typical findings allowing a diagnosis were considered to be: typical skin changes, proximal muscle weakness, MRI findings, elevated muscle enzymes, nailfold capillary changes, presence of calcinosis and muscle biopsy. Regarding induction treatment of moderate/severe JDM: 59%/74% opted for intermittent intravenous methylprednisolone (IVMP) pulse therapy, and 21%/40% for conventional high-dose oral glucocorticoids. Methotrexate (MTX) was the preferred disease-modifying conventional anti-rheumatic drug (cDMARD) for moderate and severe JDM. Regarding the management of refractory moderate or severe JDM, intravenous immune globulins, mycophenolate mofetil and rituximab were preferred treatment options.ConclusionThere is consensus about the diagnosis of JDM strongly supported by classic clinical and MRI findings. There is great variety in the treatment of JDM in Germany regarding both induction and maintenance therapy. The development of consensus-based treatment strategies for JDM based on harmonization of current clinical practice is essential in order to allow comparative effectiveness research in the future.

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review.

Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease. CyA provides a therapeutic option in psoriatic arthritis, being rather effective in skin disease. CyA is currently regarded as a second-line option for patients with inflammatory myopathies refractory to standard regimen. CyA is used even in paediatric rheumatology, in particular in the management of juvenile dermatomyositis and macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Importantly, CyA has been shown to suppress the replication of HCV, and it can thus be safely prescribed to those patients with chronic hepatitis C. Noteworthy, CyA can be administered throughout the gestation course. Surely, caution should be paid to CyA safety profile, in particular to its nephrotoxicity. Even though most evidence comes from small and uncontrolled studies with few randomised controlled trials, CyA should be still regarded as a valid therapeutic tool in 2016 rheumatology.

PReS-FINAL-2010: Predictive value of whole-body MRI in juvenile dermatomyositis

One of the major challenges in the management of juvenile dermatomyositis (JDM) is to accurately assess disease activity in order to optimize therapeutic strategies. Whole-body(WB)-MRI enables a reliable analysis of the site (skeletal muscle, subcutaneous tissue, myofascia) and magnitude of inflammatory process throughout the entire body. By providing an accurate estimation of the total disease activity load, this technique could be useful to tailor treatment according to disease severity.

An update on the diagnosis and management of juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a rare pediatric rheumatic disease. It belongs to the group of idiopathic inflammatory myopathies of childhood. JDM is the most frequent of the idiopathic inflammatory myopathies (it represents 85% of cases) among children. JDM is classified by the Bohan and Peter criteria set in 1975. New criteria are being developed and newly developed diagnostic tools are being used in daily clinical practice. This review focuses on recent publications regarding the epidemiology, pathogenesis, classification and treatment of JDM, and will discuss some relevant publications from the adult myositis literature.

Calcinosis; a Difficult Problem in Juvenile Dermatomyositis

Objective: Juvenile dermatomyositis (JDM) is a rare disease of childhood characterized by nonsuppurative inflammation of striated muscle and skin and calcinosis is one of the most important sequelae of the disease. Methods: In this study we investigated the clinical findings, complications and prognosis of 13 patients (9 girls, 4 boys) aged between 6-14 years and diagnosed to have JDM in a period of 10 years. We documented the data of the patients that had calcinosis in detail and discussed the risk factors for the development of this complication and its management. Results: Three patients (23%) developed calcinosis after 1.5-7 years during the therapy with prednisolone and one with additional methotrexate. They did not show any improvement despite aggressive therapy with anti-inflammatory drugs including prednisolone, methotrexate and colchicine together with biphophonates and diltiazem. Conclusion: Since calcinosis may develop and progress despite appropriate therapy, controlled trials including large number of patients are needed to show the optimal management of JDM to prevent the development and progression of this complication.

Whole-body MRI: a helpful diagnostic tool for juvenile dermatomyositis case report and review of the literature

Juvenile Dermatomyositis (JDM) is a rare autoimmune disease in childhood. Distinction between muscle inflammation and a residual state can be difficult especially under immunosuppressive therapy and in active disease without correlating muscle enzyme tests or functional muscle scores. Our goal is to demonstrate the benefit of whole-body magnetic resonance imaging (MRI) as a diagnostic modality in the detection and management of JDM. One patient with JDM was monitored using clinical examination, muscle enzyme tests, muscle scores and whole-body MRI. During immunosuppression, the patient presented several times to our department without clear correlation between clinical picture, muscle enzyme tests and muscle scores. Whole-body MRI proved reliable in assessing the true state of the disease, thus providing invaluable information in the management of the inflammatory myopathy. This is of utmost importance for the therapeutic optimization in order to prevent further damage especially in children with active but subclinical disease.