Juvenile dermatomyositis (JDM) is an autoimmune disease in children, which causes skin rash, tissue damage and muscle inflammation (myositis), resulting in weakening of muscles. Juvenile dermatomyositis differs from adult dermatomyositis and is not associated with increased risk of cancer. Know what is Juvenile dermatomyositis, its causes, symptoms, treatment and prognosis.

Anti-MDA-5 antibody was first discovered in a Japanese population of amyopathic dermatomyositis patients in 2005. Autoantibodies were analysed from 298 patients with various connective tissue diseases, and anti-MDA-5 antibodies were detected in 8 of 42 patients with dermatomyositis. Those with anti-MDA-5 autoantibodies had significantly more rapidly progressive interstitial lung disease (ILD) when compared with patients without anti-MDA-5 autoantibodies.1. Another retrospective study found 10 out of 77 dermatomyositis patients were positive for the anti-MDA-5 antibody. This study found a characteristic cutaneous phenotype including skin ulceration, tender palmar papules or both. Hyperkeratosis of digital pulp, ulceration located on lateral nail folds, elbows, knees and Gottron papules were significantly associated with the disease. Patients with anti-MDA-5 antibodies also had an increased risk of hand swelling, arthritis/arthralgia and diffuse hair loss.2. Our patient did not present with ...

Subcutaneous oedema as a presenting feature of polymyositis/dermatomyositis: a poor prognostic indicator? - Grand Rounds Subcutaneous oedema as a presenting feature of polymyositis/dermatomyositis: a poor prognostic indicator? - Widespread subcutaneous oedema is a rare presenting feature of polymyositis (PM)/dermatomyositis (DM). It was reported in the initial description of the disease by Wagner in 1877 but only nine cases have since been reported in the literature and it is not listed in standard textbooks of rheumatology. We present a further case of subcutaneous oedema as a presenting feature of dermatomyositis, briefly review the existing literature and postulate that this presentation represents a subset of the disease with a poorer prognosis.

Dermatomyositis is an idiopathic inflammatory disease of the muscles, in which inflammatory cells invade the skin and muscles. This leads to progressive muscle weakness at the proximal extremities, along with characteristic skin lesions.. The classification method proposed by Bohan and Peter [1] has been widely used to subtype and diagnose dermatomyositis. The diagnosis of definite dermatomyositis is made when 3 or more of the following criteria are met in addition to the presence of characteristic skin lesions: 1) symmetric weakness of proximal muscles, 2) characteristic findings on muscle biopsy, elevated serum muscle enzyme levels, and 3) abnormal electromyogram findings. The diagnosis of probable dermatomyositis is made when muscle biopsy shows findings that indicate dermatomyositis in the absence of skin lesions. Typical skin lesions suggestive of dermatomyositis include a heliotrope rash (violaceous eruption on the upper eyelids), Gottrons papules (scaly, erythematous papules at the ...

TY - JOUR. T1 - Anti-melanoma differentiation-associated protein 5-associated dermatomyositis. T2 - Expanding the clinical spectrum. AU - Hall, John C.. AU - Casciola-Rosen, Livia. AU - Samedy, Lesly Ann. AU - Werner, Jessie. AU - Owoyemi, Kristie. AU - Danoff, Sonye K.. AU - Christopher-Stine, Lisa. N1 - Copyright: Copyright 2014 Elsevier B.V., All rights reserved.. PY - 2013/8. Y1 - 2013/8. N2 - Objective Autoantibodies against melanoma differentiation-associated protein 5 (MDA-5) have been described in several Asian dermatomyositis (DM) cohorts, often associated with amyopathic DM and rapidly progressive interstitial lung disease (ILD). A recent study of a DM cohort seen at a US dermatology clinic reports that MDA-5 autoantibodies are associated with a unique cutaneous phenotype. Given the widening spectrum of clinical findings, we evaluated the clinical features of anti-MDA-5-positive patients seen at a US myositis referral center. Methods One hundred sixty DM patients were screened for ...

Dermatomyositis, Amyopathic dermatomyositis, Idiopathic inflammatory myopathies, Petges-Clejat syndrome, Adult onset dermatomyositis, Juvenile onset dermatomyositis, Paraneoplastic dermatomyositis, Dermatopolymyositis. Authoritative facts from DermNet New Zealand.

TY - JOUR. T1 - Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis. AU - Gunawardena, Harsha. AU - Wedderburn, L R. AU - Chinoy, H. AU - Betteridge, Zoe E. AU - North, Jean. AU - Ollier, W E R. AU - Cooper, R G. AU - Oddis, C V. AU - Ramanan, A V. AU - Davidson, J E. AU - McHugh, Neil J. PY - 2009/6. Y1 - 2009/6. N2 - Objective. The identification of novel autoantibodies in juvenile dermatomyositis (DM) may have etiologic and clinical implications. The aim of this study was to describe autoantibodies to a 140-kd protein in children recruited to the Juvenile DM National Registry and Repository for UK and Ireland. Methods. Clinical data and sera were collected from children with juvenile myositis. Sera that recognized a 140-kd protein by immunoprecipitation were identified. The identity of the p140 autoantigen was investigated by immunoprecipitation/immunodepletion, using commercial monoclonal antibodies to NXP-2, reference anti-p140, and anti-p155/140, ...

Background/Purpose: Juvenile dermatomyositis (JDM) is an autoimmune disease that causes vasculopathy and inflammation of skin and muscles. Previous studies in adult dermatomyositis suggest increased risks of cardiovascular disease, but such assessments in JDM remain underexplored. This study evaluates cardiovascular risk factors and outcomes in children with versus without JDM.. Methods: Data were analyzed from the 2002-2012 Nationwide Inpatient Sample, containing a representative 20% stratified sample of all hospitalizations in the United States. Primary (i.e. condition chiefly responsible for inpatient admission) vs secondary diagnoses of JDM (age ,18 years) were identified using the previously validated ICD-9-CM code 710.3. Comorbidities were identified using ICD-9-CM codes in NIS for each patient discharge. Survey weighted logistic regression models were used to determine associations of JDM with comorbidities. Multivariate models included age, sex and race/ethnicity (model-1), as well as ...

We present a 29-year-old male with a history of treatment resistant juvenile dermatomyositis (JDM). The patient was admitted for complaints of nausea, diarrhea and abdominal pain and was subsequently found to have intestinal perforation on imaging. The patient had also exhibited classic dermatologic findings alongside rare dermato-pathological manifestations of JDM on examination; likely consequences of his underlying disease process. This case serves to present these rare findings and analyze the similarities of JDM and adult dermatomyositis (DM). In addition, overall diagnosis and treatment of resistant/severe JDM is explored. High clinical suspicion alongside an interdisciplinary approach is warranted for such patients given their extensive risk factors for future complications.

Serum interferon-α is a useful biomarker in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositisSerum interferon-α is a useful biomarker in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis ...

Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in

Blood accessible biomarkers to assess disease activity and their response to therapies in Juvenile Dermatomyositis (JDM) are urgently needed. This pilot study aims to identify serum protein biomarkers associated with clinical disease activity in untreated JDM and their response to medical therapy. SomaScan® technology screened JDM patients for 1305 proteins at three points: 1) before start of treatment, 2) while on therapy, and 3) after treatment tapering when patients were clinically inactive. To define disease associated biomarkers, SomaScan® data from untreated JDM patients (n = 8) were compared to SomaScan® data from an independent age-matched healthy control group (n = 12). Longitudinal analysis defined treatment responsive proteins at three time points: untreated (7 samples), treated (7 samples), and clinically inactive (6 samples). To confirm the SomaScan® data, a subset of nine candidate proteins (CXCL11, IL-17B, IL-17D, IL-22, CXCL10, MCP-1, ANGPT2, MIF, IL-23) were tested by ELISA after

This thesis describes studies in the field of juvenile dermatomyositis (JDM), a chronic inflammatory disease in which the microvasculature is attacked by the immune system. Clinical characteristics include symptoms of muscles (e.g. weakness) and skin (e.g. Gottrons papules over the extensor joint surfaces, heliotrope rash over the eyelids, and photosensitivity). The ... read more objectives of the studies in this rare disease were: A) to gain a better understanding of the exercise intolerance and fatigue; B) to contribute to an improvement in the prognostics and monitoring of disease course; and C) to examine the feasibility, safety, and efficacy of exercise training. In a cross-sectional study, the oxygenation and hemodynamics during exercise and recovery were assessed in children with JDM with near-infrared spectroscopy and compared with results from children in juvenile idiopathic arthritis as well as healthy controls. Patients with JDM showed lower values of total hemoglobin in the vastus ...

Dermatomyositis is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness. A key symptom of dermatomyositis is a skin rash that precedes, accompanies, or follows progressive muscle weakness. The rash looks patchy, with purple or red discolorations, and characteristically develops on the eyelids and on muscles used to extend or straighten joints, including knuckles, elbows, knees, and toes. Red rashes may also occur on the face, neck, shoulders, upper chest, back, and other locations, and there may be swelling in the affected areas.. The rash sometimes occurs without obvious muscle involvement. Adults with dermatomyositis may experience weight loss, a low-grade fever, inflamed lungs, and be sensitive to light such that the rash or muscle disease gets worse. Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (called ...

It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). The aim of this study was to find clinical features most strongly associated with outcome variables in JDM as a first step towards tailor-made treatment. In a large, prospectively followed, multicenter cohort study of 340 patients with JDM, each contributing multiple visits, a Bayesian model of disease activity was developed, using the four continuous outcome variables creatine kinase (CK), childhood myositis assessment score (CMAS), manual muscle testing of 8 muscle groups (MMT8) and the physicians global assessment of disease activity (PGA). Covariates were clinical signs and symptoms. Correlations among visits of the same patient were resolved by introducing subject-specific random effects. Myalgia and dysphonia were associated with worse disease activity according to all outcome variables. Periorbital rash, rash on the trunk, rash over large joints, nail fold changes and facial swelling were

Calcinosis, a serious complication of dermatomyositis, involves deposition of calcium (carbonate apatite) in soft tissue, and can result in negative impacts on quality of life and physical function. To date, there are no known effective therapies that are approved for the treatment of dermatomyositis-associated calcinosis, and there is no consensus within the medical community on the optimum treatment strategy for this often-debilitating condition.. A few reports in the literature describe treatment successes with a variety of therapeutics; however, these data are from anecdotal reports or case series and thus provide limited scientific evidence of effectiveness. Recently published reports as well as personal observations within our group have suggested that intravenous sodium thiosulfate treatment may benefit calcinosis patients. In order to gather more robust data on the utility of this medication in the treatment of calcinosis associated with adult and juvenile dermatomyositis, we propose to ...

The treatment of dermatomyositis is directed toward the specific symptoms that are apparent in each individual and thus can vary from one patient to another. Such treatment may require the coordinated efforts of a team of medical professionals, such as pediatricians or internists; physicians who specialize in the diagnosis and treatment of connective tissue disorders (rheumatologists); specialists in the functioning of the immune system (immunologists); and/or other health care professionals. In general, treatment for the muscle involvement associated with dermatomyositis requires the use of glucocorticoids. Treatment for the skin findings associated with dermatomyositis includes: sun avoidance, sunscreens, topical glucocorticoids, anti-malarial agents, methotrexate, mycophenolate mofetil, and/or intravenous immunoglobulin (IVIg).. Glucocorticoids, particularly prednisone, are widely used in the treatment of dermatomyositis and are often used first-line. Such medications, which are similar to ...

We report a 14-year-old girl with juvenile dermatomyositis (JDM) complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day) of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually

OBJECTIVE: To report the efficacy of probenecid for calcinosis of juvenile dermatomyositis (JDM) and assess the changes in phosphorus metabolism during treatment. METHODS: Biochemical studies of calcium and phosphorus metabolism were performed in a 9-year-old girl with JDM and extensive calcifications before and during probenecid treatment. RESULTS: The calcifications resolved over 18 months of treatment. Probenecid was found to be effective in reducing calcifications by increasing renal phosphate clearance. CONCLUSIONS: The tendency for calcifications in some patients with JDM might be related to an increase in renal phosphate reclamation, and therefore, probenecid treatment may be effective in these patients. ...

Patients age greater than or equal to 18 years.. Patients with a diagnosis of definite or probable dermatomyositis by criteria of Bohan et. al., with either active rash typical of dermatomyositis, history of rash typical of dermatomyositis, or Gottrons papules.. Patients with a manual muscle testing score less than or equal to 136/170.. Patients with a disease duration greater than or equal to 6 months.. Patients with persistent disease (defined as active rash plus CK greater than or equal to 2 times ULN), or rapidly progressive disease, or response to steroids with inability to taper dose, or unacceptable side effects of steroids.. Patients may be on stable (times 28 days prior to Visit 2) dose of MTX or AZA.. No other immunosuppressive agents times 84 days prior to first dose.. Patients on stable oral steroid use for 28 days prior to Visit 2.. Patients with adequate hematologic function, defined as hemoglobin greater than or equal to 8.5 g/dl, WBC greater than or equal to 3,000 mm(3), ...

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterised by skin manifestations. Diagnosis is based on the presence of a symmetrical proximal myopathy, raised muscle enzymes, myopathic changes on electromyography, a characteristic muscle biopsy, and a typical skin rash (e.g., peri-orbital dusky violaceous erythema, or macular violaceous erythema such as in V, shawl, and Gottrons signs). [1] Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292:403-407. http://www.ncbi.nlm.nih.gov/pubmed/1089199?tool=bestpractice.com Some patients present with typical cutaneous manifestations but have delayed-onset, subclinical, or absent muscle disease. [2] Gerami P, Schope JM, McDonald L, et al. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006;54:597-613. ...

Polymyositis (PM) and dermatomyositis (DM) are two distinct subgroups of idiopathic inflammatory myopathies, a chronic inflammatory disorder clinically characterized by muscle weakness and inflammatory cell infiltrates in muscle tissue. In PM, a major component of inflammatory cell infiltrates is CD8+ T cells, whereas in DM, CD4+ T cells, plasmacytoid dendritic cells, and B cells predominate. In this study, with the aim to differentiate involvement of CD4+ and CD8+ T-cell subpopulations in myositis subgroups, we investigated transcriptomic profiles of T cells from peripheral blood of patients with myositis. Total RNA was extracted from CD4+ T cells (PM = 8 and DM = 7) and CD8+ T cells (PM = 4 and DM = 5) that were isolated from peripheral blood mononuclear cells via positive selection using microbeads. Sequencing libraries were generated using the Illumina TruSeq Stranded Total RNA Kit and sequenced on an Illumina HiSeq 2500 platform, yielding about 50 million paired-end reads per sample. Differential

Juvenile dermatomyositis (JDM) is a systemic autoimmune disease in children and adolescents characterized by a vasculopathy that primarily affects

1. Renal haemodynamics were monitored over an average period of 19 months in 17 children being treated with cyclosporin A. Sixteen had juvenile dermatomyositis and one had chronic polyneuropathy. The dose of cyclosporin A ranged from 2.3 to 8.3 mg day−1 kg−1 (median 4.1 mg day−1 kg−1).. 2. Glomerular filtration rate (expressed in terms of extracellular fluid volume), renal blood flow (expressed as a fraction of cardiac output) and filtration fraction were measured by using 99mTc-labelled diethylenetriaminepenta-acetate. They were compared with the dosage and trough blood levels of cyclosporin A, and, in 15 patients receiving prednisolone in addition to cyclosporin A, with steroid dosage.. 3. All 17 children had a renogram performed 6 months after starting cyclosporin A treatment. Nine of them also had a renogram before starting cyclosporin A treatment (baseline study), while 13, in addition to their renogram 6 months after starting cyclosporin A treatment, also had at least one further ...

Learn more about Juvenile Dermatomyositis symptoms, diagnosis, and treatments from experts at Boston Childrens, ranked best Childrens Hospital by US News.

OBJECTIVE: Assessment of myositis patients has relied on symptoms, strength testing, and serum muscle enzyme activity. Recently, functional assessments and evaluation of strength by dynamometry and of disease activity by magnetic resonance imaging ha

Dermatomyositis (DM) is inflammatory myopathy or myositis characterised by muscle weakness and skin manifestations. In the differential diagnosis of DM, the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis. Retinoic acid inducible-gene I (RIG-I), a receptor of innate immunity that promotes type I interferon, was observed in perifascicular areas in DM. This study compared the value of RIG-I expression with perifascicular atrophy as a biomarker of DM in 115 coded muscle biopsies: 44 patients with DM, 18 with myositis with overlap, 8 with ASS, 27 with non-DM inflammatory myopathy (16 with polymyositis, 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with ...

DIAGNOSIS OF DERMATOMYOSITIS AND POLYMYOSITIS A STUDY OF 102 CASES ROSANA HERMINIA SCOLA*, LINEU CESAR WERNECK**, DANIEL MONTE SERRAT PREVEDELLO***, EDIMAR LEANDRO TODERKE****, FÁBIO MASSAITI IWAMOTO***** ABSTRACT - Patients with dermatomyositis (DM) or polymyositis (PM) were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p ,0.01]. DM patients complained more arthralgia [p ,0.05], dysphagia [p ,0.03] and weight loss [p ,0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p ,0.002]. PM presented more significant increase in creatine kinase (CK) [p ,0.02] and in alanine aminotransferase (ALT) [p ,0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early ...

The mean age was 11.6 years (6-18). Subclinical mild/moderate obstruction according to American Thoracic Society criteria was observed in 35% and DLCO was reduced in 20% of JDM patients. Spirometric and/or DLCO abnormalities were observed in 45% of patients. In plethysmography, reduced total lung capacity (TLC) and conductance were observed in 25% and 50% of JDM patients, respectively. In contrast, increased resistance and residual volume (RV)/TLC were evidenced in 10% and 35% of JDM patients, respectively. Thirteen patients underwent HRCT and 8 had alterations: interstitial lung disease in 6 and a mixed pattern in two. A positive correlation was observed between DAS and ratio between forced expiratory volume in one second and vital capacity (VEF1/CV) (r=+0.50, p=0.003), conductance (r=+0.46, p=0.045) and HRCT score (r=+0.60, p=0.003). A positive correlation was observed between CMAS and VEF1/CV (r=+0.47, p=0.042), DLCO (r=+0.67, p=0,002) and 6MWT (r=+0.54, p=0.048), and negative correlation ...

It is not clearly understood what causes dermatomyositis but there are shared characteristics of this condition with other autoimmune disorders where the immune system attacks its own body tissues, mistakenly. In dermatomyositis, the small blood vessels found in muscular tissue are affected. The inflammatory cells tend to enfold these small blood vessels, which causes the degeneration of muscle fibers causing the muscles weakness.. There may be present complications in patients with dermatomyositis and they include difficulty swallowing, aspiration pneumonia, breathing problems, and calcium deposits. When the muscles around the esophagus are affected by this skin condition, one may have problem with swallowing, a condition known as dysphagia. This could cause other problems such as malnutrition and weight loss.. Moreover, the difficult in swallowing may cause one to breath liquids and foods or saliva into their lungs which causes aspiration pneumonia. When the chest muscles are affected, one is ...

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Dermatomyositis is an acquired auto-immune disease characterized by skin lesions and muscle-specific pathological features such as perifascicular muscle fibre atrophy and vasculopathy. Dermatomyositis patients display an upregulation of type I interferon-inducible genes in muscle fibres, endothelial cells, skin and peripheral blood. However, the effect of type I interferon on muscle tissue has not yet been determined. Our aim was to study the pathogenicity of type I interferon in vitro and to evaluate the efficacy of the type I interferon pathway blockade for therapeutic purposes. The activation of type I interferon in differentiating myoblasts abolished myotube formation with reduced myogenin expression while in differentiated myotubes, we observed a reduction in surface area and an upregulation of atrophy-associated genes. In vitro endothelial cells exposure to type I interferon disrupted vascular network organization. All the pathogenic effects observed in vitro were abolished by ruxolitinib. Finally

Juvenile dermatomyositis is one of the conditions in a group of conditions called the dermatomyositis/polymyositis complex. The conditions in this complex are characterized by muscle damage due to an inflammatory process of the blood vessels that lie under the skin and muscles. Skin changes around the eyelids and over the knuckles and finger joints are also seen. Juvenile dermatomyositis is the condition most often seen in children ...

Polymyositis and dermatomyositis are part of a range of conditions known as myositis-spectrum disorders. Hinsdale Orthopaedics in New Lenox, Joliet and Chicago, IL treats these autoimmune conditions.

A group of international researchers writing in Pediatric Rheumatology have proposed a plan for tapering and discontinuing glucocorticoids in patients with juvenile-onset dermatomyositis, based on changes in core set measures of disease activity in the first 6 months of treatment.“Therapeutic approaches for adult patients with [dermatomyositis] are not standardized, while those for children

Marie Lecouffe-Desprets, Caroline Hemont, Antoine Neel, Claire Toquet, Agathe Masseau, et al.. Clinical contribution of myositis-related antibodies detected by immunoblot to idiopathic inflammatory myositis: A one-year retrospective study. Autoimmunity, Taylor & Francis, 2018, 51 (2), pp.89--95. ⟨10.1080/08916934.2018.1441830⟩. ⟨hal-01881121⟩ ...

Before considering any therapeutic intervention of the autoimmune disease per se, prompt and adequate diagnosis and therapy of the underlying neoplasm in the paraneoplastic variant is mandatory. Once an underlying neoplasm is thoroughly excluded, the combined therapy consisting of aggressive sun-protection and immunomodulatory/immunosuppressive therapy should be initiated.. It is well known that the photosensitivity and cutaneous lesions in dermatomyositis are the most refractory among the connective tissue diseases. This phenomenon is most likely due to the fact that UVA is the primary culprit for the aforementioned photosensitivity. As previously mentioned, UVA is ubiquitous, goes through most glasses, is present year round, on cloudy days and in most sources of artificial lights. Furthermore, unlike UVB, UVA does not induce immediate sunburn, leading to patients with dermatomyositis not to associate the sun exposure with the cutaneous flare. All of the aforementioned facts make UVA a near ...

TY - JOUR. T1 - Dermatomyositis is associated with an increased risk of cardiovascular and cerebrovascular events. T2 - A Taiwanese population-based longitudinal follow-up study. AU - Lai, Y. T.. AU - Dai, Y. S.. AU - Yen, M. F.. AU - Chen, L. S.. AU - Chen, H. H.. AU - Cooper, R. G.. AU - Pan, S. L.. PY - 2013/5. Y1 - 2013/5. N2 - Background While the chronic inflammation related to autoimmune diseases is known to be associated with an increased cardiovascular risk, much less is known about cerebrovascular risks. Objectives The present population-based, age- and sex-matched follow-up study was undertaken to investigate the risks of acute myocardial infarction (AMI) and ischaemic stroke in patients with dermatomyositis (DMS). Methods In total 907 patients with DMS were enrolled and compared with a non-DMS control group consisting of 4535 age- and sex-matched, randomly sampled subjects without DMS. The AMI-free and ischaemic stroke-free survival curves were generated using the Kaplan-Meier ...

Results The serum PGRN levels were significantly higher in the DM patients (median: 100 ng/ml) than in the PM patients (60.4 ng/ml, P=0.0028) and NHCs (48.3 ng/ml, P,0.0001). Of the total DM patients, the levels were significantly higher in DM with A/SIP than that in DM with CIP (P,0.0001) or without ILD (P=0.0002). Proportion of clinically amyopathic DM is higher in DM with A/SIP than in DM with CIP or without ILD (76.9%, 40%, and 5.9%, respectively). The serum PGRN levels correlated significantly with serum ferritin (rs=0.71, P=0.0001), LDH (rs=0.59, P=0.0003), and CRP (rs=0.57, P=0.0005) levels. They significantly decreased following successful treatment of DM (P=0.0313). Moreover, the cumulative survival rate for 6 months was significantly lower in the group with serum PGRN levels , or =200 ng/ml (60%) than that in the group with serum PGRN levels , 200 ng/ml (P=0.001).. ...

Demographic, clinical, and laboratory features that predict underlying malignancy in patients with dermatomyositis (DM) are poorly known. We conducted a retrospective study in all adult patients with a definite (n = 75) or probable (n = 32) diagnosis of DM according to Bohan and Peter criteria or with amyopathic DM (n = 14) who were referred to 2 departments during a 13-year period. The diagnosis of malignancy-associated DM was retained if DM occurred in a context of recently diagnosed malignancy or if a malignancy was diagnosed during the 5 years following the diagnosis of DM. The Kaplan-Meier method was used to assess the cumulative incidence rates of underlying malignancy during the first 5 years of DM. Factors associated with malignancy in patients with DM were identified by Cox proportional hazards models. During the study period, 121 patients fulfilled the inclusion criteria (median age, 52 yr; range, 19-77 yr; women: 70%). For 29 of them, the diagnosis of malignancy-associated DM was retained.

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Results Among 20 randomized patients (9 DM, 11 PM; 13 female, 7 male), 17 were included in the analyses and 8 (47%) achieved the DOI after 6 months of active treatment. No differences between DM and PM, or female and male patients could be seen. At 3 months after study start, 5 of the 10 (50%) patients in the active treatment arm were responders achieving the DOI compared to only 1 of the 7 (14%) patients in the delayed onset arm.. After active treatment for 6 months (n=17), significant improvement was seen in muscle strength, assessed by the manual muscle test (MMT)-8, from (median) 70 to 73 (p=0.0082), in gastrointestinal disease activity from 3 to 0 (p=0.0156), and in muscle disease activity from 18 to 10 (p=0.0133). SF-36 physical was significantly improved from median 31 at start to 36 at end of treatment (p=0.0054). There were 36 adverse events (AE) reported. Eight AE were judged as related to the drug, of which 4 were mild and 4 were moderate. These included infections, flank pain and ...

Dermatomyositis is a rare autoimmune disease. Its characterised by a rash, which has a number of appearances, including something called gottrons papules and associated muscle weakness and pain. Doesnt sound too bad when you write it down like that does it...truth is, it can be debilitating and cause disability and even death. There is a form…

1. Drug-induced myositis (myositis in patients taking medications known to induce myositis-like syndromes, including, but not limited to, statin agents, fibric acid derivatives, and colchicine). 2. Juvenile polymyositis; inclusion body myositis; cancer-associated myositis, defined as the diagnosis of myositis within 2 years of the diagnosis of cancer except basal or squamous cell skin cancer or carcinoma in situ of the cervix if at least 5 years since excision. 3. Myositis in overlap with another connective tissue disease (CTD) that precludes the accurate assessment of a treatment response (for example, difficulty in assessing muscle strength in a scleroderma patient with associated myositis) 4. History of receiving a live vaccine 4 weeks prior to initiation of study treatment. 5. Joint disease, severe calcinosis, or other musculoskeletal condition, which precludes the ability to quantitate muscle strength. 6. Wheelchair bound patients. 7. Known hypersensitivity to abatacept or prior receipt of ...

These findings are consistent with the idea that plasmacytoid DCs are mediators of muscle inflammation in juvenile DM. The abundance of CD83+ plasmacytoid DCs in perivascular areas and the overexpression of CCL19 and CCL21 in perivascular cellular foci suggest that in situ activation and maturation …

Classic dermatomyositis (DM) is an idiopathic inflammatory myopathy that most commonly presents with progressive, symmetric proximal muscle weakness and a group of characteristic cutaneous findings. The cutaneous manifestations of DM may also develop

Damage is common in myositis after a median duration of 5 years in patients with adult-onset disease and 6.8 years in patients with juvenile-onset disease. The MDI has good content, construct, and predictive validity in juvenile and adult myositis.

Objective. To characterize serum autoantibody profiles of patients with idiopathic inflammatory myopathies (IIM) by searching for myositis-specific (MSA) and myositis-associated (MAA) antibodies with sensitive and specific laboratory tests. Methods. We tested the sera from 46 Caucasian patients diagnosed as affected with IIM at the Division of Rheumatology of Padova University (21 polimyositis, PM; 22 dermatomyositis, DM; 3 myositis overlap syndrome). All patients had definite IIM according to the criteria of Bohan-Peter. MSA including anti-tRNA synthetase (anti-Jo-1 and others) and anti-Mi-2 were determined by RNA immunoprecipitation and a modified immunoblot test, respectively. MAA (-U1RNP, -U2RNP, RoRNP, PM/Scl, Ku) were detected by counterimmunoelectrophoresis and immunoblot. Results. Serum MSA and/or MAA were found in 30/46 (65%) patients with IIM. Twenty-three patients (50%) were positive for at least one MSA: anti-Jo-1 in 15 (33%), anti-Mi-2 in 6 (13%), and other anti-tRNA synthetase in 3 ...

TY - JOUR. T1 - Dermatomyositis and polymyositis associated with malignancy. T2 - A 21-year retrospective study. AU - András, C.. AU - Ponyi, A.. AU - Constantin, T.. AU - Csiki, Zoltán. AU - Szekanecz, Éva. AU - Szodoray, Peter. AU - Dankó, K.. PY - 2008/3. Y1 - 2008/3. N2 - Objective. To analyze clinical and laboratory data of patients diagnosed with dermato- or polymyositis between 1985 and 2006, retrospectively, with particular emphasis on association with malignant diseases. Methods. A thorough clinical assessment was performed on the immunological features and therapeutic responses, as well as survival data. In the case of 155 myositis patients, HLA haplotypes were also investigated. Results. Out of 309 patients with myositis in our database, malignant disease was found in 37 cases. Thirty patients had dermatomyositis (28.8%), and 7 had polymyositis. In 64.8% of the cases, the malignancy and myositis appeared within 1 year. The highest probability for tumor recognition was before 2 ...

NASCIF, Ana K. S. et al. Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data. J. Pediatr. (Rio J.) [online]. 2006, vol.82, n.1, pp.40-45. ISSN 0021-7557. http://dx.doi.org/10.2223/JPED.1435.. OBJECTIVE: Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. Our aim was to correlate disease activity (clinical and laboratory data) and nailfold capillaroscopy findings in 18 patients with inflammatory myopathies. METHODS: This prospective study included 13 juvenile dermatomyositis patients (Bohan and Peter criteria) (mean age of 8.8 years) and five patients with overlap syndrome (mean age of 15.7 years). We evaluated disease activity (skin abnormalities and muscle weakness, muscle enzymes and acute phase ...

Sarcomas are rare and heterogeneous tumours of mesenchymal origin, with over 100 histological subtypes. Paraneoplastic dermatomyositis has rarely been described in sarcoma. This is the first documented case of paraneoplastic dermatomyositis in a patient with metastatic leiomyosarcoma. A 43-year-old female diagnosed with metastatic leiomyosarcoma of unknown primary presented with a mild rash in sun-exposed areas of her face and upper chest, with no other neuromuscular symptoms. This rash resolved with systemic treatment with doxorubicin for metastatic leiomyosarcoma. Imaging assessment confirmed overall stable disease after chemotherapy completion. She presented acutely 2 months later with new onset rash in a shawl-like distribution, periorbital oedema and proximal muscle weakness. Based on the characteristic cutaneous signs and symmetrical proximal muscle weakness, abnormal electromyography and raised skeletal muscle enzymes with a positive anti-transcription intermediary factor-1 gamma antibody result,

If you suffer from Dermatomyositis, we could help you to find specialist life insurance, critical illness cover and income protection policies tailored to your needs.

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Looking for online definition of garden heliotrope in the Medical Dictionary? garden heliotrope explanation free. What is garden heliotrope? Meaning of garden heliotrope medical term. What does garden heliotrope mean?

Calcinosis -A condition in which calcium salts are deposited in various body tissues. In juvenile dermatomyositis, calcinosis usually takes the form of small lumps of calcium compounds deposited in muscles or under the skin. Contracture -A tightening or shortening of muscles that prevents normal movement of the associated limb or other body part. Coxsackie virus -A type of enterovirus that may produce a variety of illnesses, including upper respiratory infections, myocarditis, and pericarditis. Coxsackieviruses resemble the virus that causes polio. Cutaneous -Pertaining to the skin. Disease-modifying anti-rheumatic drugs (DMARDs) -A group of medications given to treat severe cases of arthritis, JDMS, and other diseases that affect the joints. All DMARDs work by modifying the immune system. Dysphagia -Difficulty in swallowing. Heliotrope -A plant ( Heliotropium arborescens ) related to borage that has lavender or deep violet flowers. The characteristic skin rash of JDMS is sometimes called a ...

Thighs subcutaneous and myofascial signal abnormalities were detected in 8/43 (18.6%) and in 12/43 (27.9%) patients respectively. WB-MRI revealed inflammatory involvement of myofascial and subcutaneous areas other than the thigh in 8/43 (18.6%) and 10/43 (23.2%) patients, respectively Concordance between WB and thigh MRI scores in the evaluation of myofascial and subcutaneous tissue was moderate (myofascial scores rs = 0.59, subcutaneous scores rs = 0.69). Although concordance in detecting muscle inflammation between thigh and WB-MRI muscle scores was excellent (rs = 0.97), two patients with negative thigh-MRI showed muscle signal abnormalities in muscle groups different from the thigh. Inter-reader agreement was excellent for both thigh-MRI and WB-MRI scores (ICC 0.96 and 0.98 respectively). Both scores showed excellent correlations with clinical measures of disease activity (Manual Muscle Test (MMT) rs = -0.82, Childhood Myositis Assessment Scale (CMAS) rs = -0.83 for thigh-MRI; MMT rs = ...

Myositis-specific autoantibodies (MSAs) are found in almost half the patients with an idiopathic inflammatory myopathy (IIM). Several clinical and epidemiological studies have suggested that MSAs are associated with specific clinical characteristics. Some of these associations are well-defined and are of clinical significance ( eg, anti-Jo-1 and the anti-synthetase syndrome), others are less well established and can cause unnecessary anxiety for both patients and physicians ( eg, anti-SRP and cardiac involvement). In this review, an overview is given of the various MSAs, their biochemical background, their clinical usefulness, and the promises they hold for a better understanding of IIM ...

There is very little data available regarding health-related quality of life (HRQoL) in rare diseases, including idiopathic inflammatory myopathy (IIM). To look at this issue further, abstract 207 (Friday Poster session) sought to investigate the magnitude of impairment in HRQoL, measured using the Medical Outcomes Trust Short Form-36 (SF-36), and the clinical correlates of physical HRQoL in early IIM.. Leclair and her colleagues at McGill University studied a group of patients inthe Inflammatory Myopathy Study cohort. Thirty-eight IIM patients were enrolled, the majority women with a mean disease duration of 1.3 +/- 1.5 years. Subtypes of IIM included dermatomyositis (43%), clinically amyopathic dermatomyositis (19%), connective tissue disease-associated myositis (24%), polymyositis (11%) and cancer-associated myositis (3%). Subjects underwent standardized clinical histories, medical examinations, and self-administered questionnaires at time of entry. Multiple linear regression was used to ...

Looking for heliotropes? Find out information about heliotropes. or name for any plant that turns to face the sun, especially members of the genus Heliotropium of the family Boraginaceae. The garden heliotrope is a... Explanation of heliotropes

Polymyositis mediated by T lymphocytes that express the gamma/delta receptor. Risk of cancer in patients with dermatomyositis or polymyositis: a population-based study

Dermatomyositis and polymyositis are long-term inflammatory muscle diseases, causing muscle weakness and disability. For some reason, the bodys immune system turns against its own muscles in an autoimmune response. Corticosteroids are the principal treatment but due to side effects, there is a need for additional treatment with drugs that suppress the immune system (immunosuppressants) or modify it (immunomodulatory therapies) to improve patient outcomes. For this review, an update of a review first published in 2005, we found ten randomised trials available, involving 258 participants.. Amongst the six studies comparing immunosuppressant with placebo, one study, investigating intravenous immunoglobulin (IVIg), showed statistically significant improvement in scores of muscle strength in the IVIg group over three months. Another study investigating etanercept showed some evidence of a longer median time to relapse in the etanercept group, a secondary outcome in this review, but no improvement in ...

The field of autoantibodies related to immune-mediated inflammatory myopathies has expanded in recent years and there is now a host of antibodies that have relevance to these myopathies. The 1975 Bohan and Peter criteria for the classification of immune-mediated inflammatory myopathies do not reflect many newer insights, and several newer classification schemes exist, but none enjoy uniform acceptance.12 Some controversy remains as to the pathophysiology behind dermatomyositis, but this disease is probably the most consistently defined. Conversely, polymyositis has several varied definitions, and in the Bohan and Peter criteria it was not delineated from inclusion body myopathy (IBM). The antisynthetase syndrome associated with antibodies described in this section does not cleanly sort under either the dermato- or polymyositis labels. The inflammatory myopathies associated with SRP and 200/100 antibodies do not even necessarily have the inflammatory muscle infiltrates that we traditionally ...

The Childhood Myositis Assessment Scale (CMAS).The Clinical Knowledge Manager is a system for collaborative development, management and publishing. It enables the implementation of knowledge governance within and across the health enterprise. Resources include archetypes, templates, terminology subsets, artefact release sets, metadata relating to clinical models and related resources. Powered by Ocean Informatics.

For more information regarding Bristol-Myers Squibb Clinical Trial participation, please visit www.BMSStudyConnect.com Inclusion Criteria: -Diagnosis of IIM based on the Bohan and Peter classification criteria: i) Subjects with dermatomyositis (DM) must also have a confirmed myositis-associated rash (Gottrons papules or a heliotrope rash preferably confirmed by skin biopsy) and 2 or more of the remaining 4 criteria. ii) Subjects with a diagnosis of IIM other than DM include PM, autoimmune necrotizing myopathy, myositis in association with another connective tissue disease (overlap myositis) and juvenile myositis subjects above the age of 18. These subjects must have a prior muscle biopsy diagnostic for IIM or a positive test for at least one myositis-specific autoantibody (anti-aminoacyl-tRNA synthetases (Jo-1, PL-7, PL-12, EJ, OJ, KS, Zo, YRS), anti-Mi-2, anti-SRP, anti-TIF1-g, anti-NXP-2, anti-MDA5, anti-SAE, anti-HMGCR). For subjects with overlap myositis, the myositis must be the principal ...

The morphological, immunohistochemical, and immunopathological analyses of muscle biopsy are essential for the diagnosis of idiopathic inflammatory myopathies (IIMs). However, they are also one of the most common causes of misdiagnosis. Although several diagnostic criteria have been proposed for the diagnosis of IIMs, misdiagnosis still remains common in clinical practice. The present study aims to characterize the inflammatory profile of IIMs, including the expression of MHC-I, MHC-II, MAC and infiltrating cells. We also investigated the sensitivity and specificity of MHC-I and MHC-II immunostaining for the diagnosis of IIMs. We found that the expression of MHC-I and MHC-II was both higher in IIMs than in non-inflammatory myopathies (NIMs). The distribution of MHC-I in IIMs is different from that of MHC-II. MHC-I is mainly located in the sarcoplasms, while MHC-II is located mostly on the sarcolemmas. Moreover, our findings suggest that MAC may be a potential marker to diagnose DM, and the ...

This website is provided for educational and informational purposes only. Cure JM is not engaged in rendering medical advice or professional services and this information should not be used for diagnosing or treating a health problem. The site author and the content providers make no representation or warranties, expressed or implied. Providing links to other websites does not imply that Cure JM endorses the information or services provided on those websites. The organizations operating those websites are solely responsible for the content found on their websites ...

• Progressive interstitial fibrosis with roentgenographic honeycombing developed in the case of a psoriatic patient who had been on a regimen of methotrexate fo

Each year at this time, we commemorate the estimated 300,000 children and their families in the United States who face the everyday challenges of living with juvenile arthritis (JA) and related diseases. Juvenile arthritis is an umbrella term used to describe the many autoimmune and inflammatory conditions or pediatric rheumatic diseases that can develop in children and teens.. The various types of juvenile arthritis share many common symptoms, like pain, joint swelling, redness and warmth, but each type of JA is distinct and has its own unique characteristics and how it affects the body.. Common Types of Juvenile Arthritis. Juvenile idiopathic arthritis (JIA). Considered the most common form of childhood arthritis, JIA includes six subtypes: oligoarthritis, polyarthritis, systemic, enthesitis-related, juvenile psoriatic arthritis or undifferentiated.. Juvenile dermatomyositis. An inflammatory disease, juvenile dermatomyositis causes muscle weakness and a skin rash on the eyelids and ...

Definition of progressive interstitial pneumonia in sheep in the Legal Dictionary - by Free online English dictionary and encyclopedia. What is progressive interstitial pneumonia in sheep? Meaning of progressive interstitial pneumonia in sheep as a legal term. What does progressive interstitial pneumonia in sheep mean in law?

Some types of autoimmune disease attack the muscles of the body. The myositis-specific antibodies (MSA) can assist in the diagnosis of polymyositis and dermatomyositis in those patients who have the diseases. About 50% of patients with polymyositis or dermatomyositis have specific MSA or myositis associated antibodies (MAA). MSA are almost never found in patients without myositis, even if they have other muscle diseases of autoimmune diseases. This means that when the physicians examination and initial testing suggest the possibility of polymyositis or dermatomyositis, a positive test for an MSA can be strong supporting evidence for the diagnosis.. For a long time the testing for these antibodies was only available in research studies, but it is now possible to obtain this testing clinically. The Myositis Profile* performed at the OMRF Clinical Immunology Laboratory includes tests for 12 of the MSAs and MAAs. Additional antibodies may be detectable using this profile. Dr. Ira Targoff is the ...

Background. To describe our myositis cohort in-depth.. Methods. From January 2006 to December 2018, all newly diagnosed myositis patients were retrospectively enrolled in the study. We performed a subtype reclassification using the 2017 EULAR/ACR criteria, following the example of the EuroMyositis registry. Disease activity and damage were measured by the newest standardized assessment-tools for clinical studies. Comparisons between myositis subgroups were conducted using Fishers exact test.. Results. We enrolled 32 patients (25 were female): six patients with dermatomyositis, six with polymyositis, eleven with overlap myositis, six with antisynthetase syndrome, one with autoimmune necrotizing myopathy, one with juvenile antisynthetase syndrome and one with juvenile dermatomyositis. The overall median follow-up period was 23-months (9-44.75). Interstitial lung disease (ILD) was most frequently seen in patients with antisynthetase syndrome. Patients with overlap myositis were more likely to have ...

Dermatomyositis, Neck Mass, Vocal Cord Paralysis Symptom Checker: Possible causes include Papillary Thyroid Carcinoma, Medullary Thyroid Carcinoma, Bronchogenic Carcinoma. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.

TY - JOUR. T1 - Effective induction therapy for anti-SRP associated myositis in childhood. T2 - A small case series and review of the literature. AU - Binns, E L. AU - Moraitis, Elena. AU - Maillard, S. AU - Tansley, S. AU - McHugh, N. AU - Jacques, Thomas S. AU - Wedderburn, L R. AU - Pilkington, Clarissa. AU - Yasin, S A. AU - Nistala, Kiran. AU - UK Juvenile Dermatomyositis Research Group (UK and Ireland). PY - 2017/10/31. Y1 - 2017/10/31. N2 - BACKGROUND: Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation.CASE PRESENTATIONS: Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash. Histology ...

Information on Idiopathic inflammatory myopathy, which may include symptoms, causes, inheritance, treatments, orphan drugs, associated orgs, and other relevant data.

Idiopathic inflammatory myopathies represent a heterogeneous group of autoimmune diseases with systemic involvement. Even though numerous specific autoantibodies have been recognized, they have not been included, with the only exception of anti-Jo-1, into the 2017 Classification Criteria, thus perpetuating a clinical-serologic gap. The lack of homogeneous grouping based on the antibody profile deeply impacts the diagnostic approach, therapeutic choices and prognostic stratification of these patients. This review is intended to highlight the comprehensive scenario regarding myositis-related autoantibodies, from the molecular characterization and biological significance to target antigens, from the detection tools, with a special focus on immunofluorescence patterns on HEp-2 cells, to their relative prevalence and ethnic diversity, from the clinical presentation to prognosis. If, on the one hand, a notable body of literature is present, on the other data are fragmented, retrospectively based and collected

Interferon induced with helicase C domain 1 (IFIH1) protein is a member of a group of RNA helicases, characterized by the conserved motif Asp-Glu-Ala-Asp (DEAD), known as DEAD box proteins. IFIH1 is also known as melanoma differentiation-associated protein 5 (MDA5), clinically amyopathic dermatomyositis autoantigen 140 kDa, helicase with 2 CARD domains (HLCD), helicard, murabutide down-regulated protein, RIG-I-like receptor 2 (RLR-2), CADM-140 autoantigen, RNA helicase-DEAD box protein 116 (RH116), DEAD/H (Asp-Glu-Ala-Asp/His) box polypeptide, and IDDM19. IFIH1/MDA5 plays a major role in sensing viral infection and in the activation of a cascade of antiviral and inflammatory responses. IFIH1 gene expression is upregulated in response to treatment with beta-interferon and a protein kinase C-activating compound, mezerein. Treatment with both these agents causes irreversible reprogramming of melanomas, while treatment with either agent alone only achieves reversible differentiation. Mutations in ...

Interferon induced with helicase C domain 1 (IFIH1) protein is a member of a group of RNA helicases, characterized by the conserved motif Asp-Glu-Ala-Asp (DEAD), known as DEAD box proteins. IFIH1 is also known as melanoma differentiation-associated protein 5 (MDA5), clinically amyopathic dermatomyositis autoantigen 140 kDa, helicase with 2 CARD domains (HLCD), helicard, murabutide down-regulated protein, RIG-I-like receptor 2 (RLR-2), CADM-140 autoantigen, RNA helicase-DEAD box protein 116 (RH116), DEAD/H (Asp-Glu-Ala-Asp/His) box polypeptide, and IDDM19. IFIH1/MDA5 plays a major role in sensing viral infection and in the activation of a cascade of antiviral and inflammatory responses. IFIH1 gene expression is upregulated in response to treatment with beta-interferon and a protein kinase C-activating compound, mezerein. Treatment with both these agents causes irreversible reprogramming of melanomas, while treatment with either agent alone only achieves reversible differentiation. Mutations in ...

In addition to self-limited myotoxicity, statins have recently been shown to trigger an immune-mediated necrotizing myopathy (IMNM), which is distinguished from polymyositis (PM) and dermatomyositis (DM) by the absence of primary inflammation on muscle biopsy. Previously, we have shown that patients with statin-associated necrotizing myopathy express an autoantibody targeting 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR), the pharmacological target of statins (1,2). We conducted a case-control study to characterize the comorbidities and detailed individual statin history and to investigate the possible clinical associations that could contribute to the development of anti-HMGCR-positive myopathy in statin-treated patients.. Within our longitudinal cohort, we identified 58 anti-HMGCR-positive statin-exposed myositis patients and 37 comparator myositis patients who were exposed to a statin and were anti-HMGCR negative. All patients met the Bohan and Peter criteria for PM or DM (3,4). Of note, ...

Monitoring Editor: Marianne Bronner-Fraser Genetically engineered mice (Myf5(nLacZ/+), Myf5(GFP-P/+)) allowing direct muscle satellite cell (SC) visualization indicate that, in addition to being located beneath myofiber basal laminae, SCs are strikingly close to capillaries. After GFP(+) bone marrow transplantation, blood-borne cells occupying SC niches previously depleted by irradiation, were similarly detected near vessels, thereby corroborating the anatomical stability of juxtavascular SC niches. BrdU pulse-chase experiments also localize quiescent and less quiescent SCs near vessels. SCs, and to a lesser extent myonuclei, were nonrandomly associated with capillaries in humans. Significantly, they were correlated with capillarisation of myofibers, regardless to their type, in normal muscle. They also varied in paradigmatic physiological and pathological situations associated with variations of capillary density, including amyopathic dermatomyositis, a unique condition in which muscle capillary loss

TY - JOUR. T1 - Correlation of antisynthetase antibody levels with disease course in a patient with interstitial lung disease and elevated muscle enzymes. T2 - Quantitation of antiglycyl tRNA synthetase antibodies by immunoprecipitation. AU - Stojanov, Lovorka. AU - Satoh, Minoru. AU - Hirakata, Michito. AU - Reeves, Westley H.. PY - 1996/4. Y1 - 1996/4. N2 - Antiglycyl tRNA synthetase is an unusual autoantibody specificity associated with polymyositis and dermatomyositis complicated by interstitial lung disease. We report here autoantibodies to glycyl tRNA synthetase in a patient with systemic lupus erythematosus and interstitial lung disease. During the course of her disease, the patient developed elevated muscle enzymes and worsening pulmonary function. A quantitative immunoprecipitation technique was developed to evaluate the relationship between autoantibody production and clinical manifestations in this patient. Increasing serum antiglycyl tRNA synthetase antibody levels correlated with ...

Background/Purpose: Myositis specific antibodies (MSA) represent not only important diagnostic tools, but also help stratify myositis patients with particular clinical features, treatment responses and disease outcomes. These antibodies also have the potential to be used in classification criteria. Consequently, standardization of MSA is of high importance. Many laboratories rely on immunoprecipitation (IP) for the detection of MSA which, however, are met with logistic, standardization, and regulatory challenges. Therefore, reliable alternatives to IP are mandatory. The objective of this study was to compare the results obtained from different assays for the detection of anti-Mi-2 antibodies. Methods: The study included 82 patients (68 females/14 males), most of whom had dermatomyositis (DM, n=57), followed by polymyositis (PM, n=16) and juvenile DM (n=9). All samples were tested using a novel particle-based multi-analyte technology (PMAT, Inova Diagnostics, research use only; Mi-2b, OJ, TIF1y, ...

|h2|Heliotrope - Cherry Pie|br/|Botanical Name: |em|Heliotropium aborescens |/em||/h2||p|Heliotrope Cherry Pie is an evergreen shrub growing from 60-100cm high and sprawling up to 2 meters wide in good conditions. It may not reach this size in every gar

Created to raise public awareness of Juvenile Myositis and to fund research for a cure for JM, including Juvenile Dermatomyositis and Juvenile Polymyositis. Find disease details, news stories, research, and a message board. ...

Intravenous infusion: Dose of 500-750 mg/square m repeated monthly for 3-6 months. An alternative regimen for SLE nephritis, termed the Euro-Lupus regimen, is 500 mg IV every 2 weeks for a total of 6 doses followed by maintenance therapy with azathioprine or mycophenolate. Whatever the regimen, adjust dose according to clinical response and WBC nadir and recovery. In many conditions, after an induction period of infusions for 3-6 months an alternative therapy is instituted to maintain remission.. Indications: Vasculitis, rheumatoid vasculitis, SLE with organ involvement, polymyositis/dermatomyositis refractory to treatment. Mechanism of Action: Interferes with DNA synthesis by alkylating and cross-linking DNA strands. Contraindications: Hypersensitivity to cyclophosphamide; pregnancy. Precautions: Bone marrow suppression, active infection, decrease dose in renal/hepatic impairment, avoid BCG and live virus vaccines, consider dose reduction if renal or hepatic function impaired, reliable ...

I Trained at Sheffield Medical School and qualified in 1995. I have worked at Sheffield, Sydney, Liverpool, Bristol and Great Ormond Street Childrens hospitals. I was involved in research into juvenile dermatomyositis(JDM) at the institute of child...

I was just recently dx with DM, I was wondering if I am the only one on here. I have currently taking prednisone 30 mg,methotrexate 2.5 x 6 weekly, doxepin 100...

On December 17, 2002 the organization changed its name from the Collie Club of America Foundation to the Collie Health Foundation in order to give the Foundation a unique identity separate from the Collie Club of America and more clearly represent our mission. ...

Inflammatory myositis is a systematic autoimmune disease that causes inflammation of muscle tissue, especially the muscles that control the movement...

A 30 year old female patient presented with complaints of weakness of all four limbs, swelling and pain on movement, difficulty in walking since last one month. She was investigated and diagnosed as a case of inflammatory myositis, she was treated with steroids, she showed good response to steroids and was discharged.. ...