Dermatomyositis

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases that share some symptoms such as muscular weakness and inflammation of skeletal muscle. Complete recovery of muscle function with pharmacological treatment does not always occur, suggesting that physical inability is a great concern for these patients. In this context, it has been speculated that physical exercise could result in functional benefits to patients with IIM, leading to an improvement in quality of life. In fact, recent studies of polymyositis (PM) and dermatomyositis (DM) support the notion that exercise training improves or at least stabilizes muscle strength and functional ability without inducing disease flares. Importantly, these benefits were observed not only during the chronic phase, but also in the course of active disease. This positive effect was found to be long term, as demonstrated by a six-month significant improvement in exercise capacity and strength. Together, these findings indicate that a well controlled exercise program can be recommended for patients with DM and PM. The optimal exercise modality training and the underlying mechanism for this encouraging response remain to be determined in future studies.

Polymyositis (PM) and dermatomyositis (DM) are connective tissue diseases (CTD) characterized by proximal muscle weakness along with changes in various internal organs, with the lungs most frequently involved. Presentation of the disease in the lungs comprises diffuse alveolar haemorrhage due to vasculitis and interstitial lung disease (ILD), which is the most frequent manifestation of CTD in the lungs and worsens the outcome and prognosis. The mechanisms involved in the ILD are not fully known, but the role of autoimmune response is unquestioned. No relationship between the severity of CTD and the changes in the lungs was observed. ILD may present at any time in the course of CTD, sometimes before the signs and symptoms of myositis occur. The more accurate imaging methods are, the more frequently changes in the lungs are detected. High resolution computed tomography (HRCT) is a gold standard in ILD imaging. Treatment of PM/DM-related ILD relays on systemic glucocorticosteroids as t...

Objective. To assess the reliability and validity of the Disease Activity Score (DAS), an instrument used to evaluate children with juvenile dermatomyositis (JDM). Methods. Psychometric study of internal consistency, reliability, rater agreement, and the relationship with measures of muscle strength and disability was conducted. Results. The DAS ratings are internally consistent (reliability ‫؍‬ 0.89) and describe a wide range of disease activity. The pediatric rheumatologists in this study agree on the presence of most of the disease indicators. Their disagreements tend to cancel each other, resulting in highly correlated (r ‫؍‬ 0.79) overall measures across raters. Estimates of muscle weakness using the DAS and ratings of muscle strength obtained independently from therapists are highly related (r ‫؍‬ ؊0.77), but estimates of disease activity and disability are weakly related (r ‫؍‬ 0.20).

Juvenile dermatomyositis, the most common infl ammatory myopathy of childhood, is a rare systemic autoimmune vasculopathy that is characterised by weakness in proximal muscles and pathognomonic skin rashes. The length of time before the initiation of treatment aff ects presenting symptoms, laboratory measures, and pathophysiology. It also aff ects disease outcomes, including the development of pathological calcifi cations, which are associated with increased morbidity. Both genetic and environmental risk factors seem to have a role in the cause of juvenile dermatomyositis; HLA B8-DRB1*0301 ancestral haplotype is a strong immunogenetic risk factor, and antecedent infections and birth seasonality suggest that environmental stimuli might increase risk. Activation of dendritic cells with upregulation of genes induced by type-1 interferon (α) in muscle and peripheral blood seems to be central to disease pathogenesis. Treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents. Disease outcome, if treatment is initiated early, is generally good. Randomised controlled trials are needed to defi ne the most eff ective treatments.

Abstract: Interstitial lung disease (ILD) is a serious complication in dermatomyositis (DM) and polymyositis (PM). In Japan, patients with DM/PM develop acute life-threatening ILD with high frequency.

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Abstract:  A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.

This article refers to papulosquamous diseases that are seen in the elderly. Many of these diseases are complex to diagnose due to the fact that they are difficult to identify, because they may resemble a similar disorder, which can be misleading. The problem that is frequently seen with these diseases is that they are commonly misdiagnosed. In this article the authors include information that will help with identifying these diseases. The authors also provide important laboratory exams that can be performed and can help guide diagnosis, as well as differential diagnosis and treatment options.

Autoimmune thyroid disease (AITD) has been associated with other autoimmune diseases such as chronic urticaria, insulin-dependent diabetes mellitus, Sjøgren's syndrome, inflammatory bowel disease, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosus, psoriasis, ankylosing spondylitis, and dermatomyositis (DM) (1,2). AITD is a common disorder affecting primarily women, and both genetic and environmental factors are included in its pathogenesis (3). DM is considered an autoimmune disease of the muscles and skin. Although AITD is the most common cause of hypothyroidism, to the best of our knowledge, only three cases of DM and AITD in the same patient have been reported in the last 40 years (4-6). We consider that both are developed from the same autoimmune background. Herein, we present a case of a 30-year-old man with a 4-year history of AITD who was diagnosed as suffering as well from DM. Ó 2006 IMSS. Published by Elsevier Inc.

Cutaneous features in the scalp area among adult patients are rarely considered as a manifestation of scabies. Three patients with clinical and laboratory data of dermatomyositis with scalp involvement (fulfill three or four of Bohan and Peter's criteria), of 4 years, 8 months, and 3 years duration, were seen at our department between 1995 and 1996. For relapses of ordinary scabies, they were treated repeatedly with local scabicide with temporary effect. After a symptom-free period during the treatment of dermatomyositis with corticosteroids and azathioprine, they developed diffuse redness with scales and crusts on the scalp areas. Light microscopy examination of material taken from these crusts showed an abundance of live mites. All patients were successfully cured of scabies with a twice oral dose of 200 microg/kg ivermectin within 8 days. Our patients with crusted scabies of the scalp and dermatomyositis prompted us to change our standard diagnostic and therapeutic regimens. ...

syndrome (pemphigus erythematosus): immunofluorescent studies in a patient. Arch Dermatol. 1970;101(6):642-645. 9. Tan EM. Special antibodies for the study of systemic lupus erythematosus: an analysis. Arthritis Rheum. 1982;25(7):753-756. 10. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997; 40(9):1725.

Methods. Nine patients with chronic PM or DM (median age 53 years, range 44 -61) were included. Assessments of impairment (10 -15 voluntary repetition maximum [VRM], the Functional Index 2 [FI-2], the Grippit, and pain rated on the Borg CR-10 scale), activity limitation (Myositis Activities Profile), and participation restriction (patients' disease impact on well-being) were performed 4 weeks prior to baseline, at baseline, and after 7 weeks of exercise. A 6-item core set of disease activity measures was administered and muscle biopsy samples of vastus lateralis were obtained at baseline and after 7 weeks of exercise. Response criteria at an individual level were set for disability and disease activity. The patients exercised 3 days per week for 7 weeks on loads allowing 10 VRM. Results. On a group level there were no significant differences between assessments at 4 weeks before baseline compared with baseline. The group improved significantly regarding 10 -15 VRM and FI-2 at 7 weeks compared with baseline (P < 0.05). All patients were responders with respect to impairment and 2 were activity limitation responders whereas participation restriction remained unchanged in all. Two patients were responders with reduced disease activity and no patient had signs of increased muscle inflammation in the muscle biopsy sample after 7 weeks of exercise. Conclusion. Patients with chronic, stable PM and DM can perform this intensive resistive exercise program with beneficial effects on impairment and activity limitation without increased muscle inflammation.

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of juvenile dermatomyositis, generalized edema has been reported rarely. In this article, we report a 14-year-old boy with juvenile dermatomyositis presenting with generalized edema. Of the diagnostic criteria of JDM, severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum muscle enzymes and myopathic electromyographic abnormalities were observed. Magnetic resonance imaging (MRI) of the lower extremities and pelvis showed marked diffuse edema in the subcutaneous tissue, muscles and myofascia. We suggest that MRI findings, which are not among the diagnostic criteria, may also be included in the diagnostic criteria of JDM. To the best of our knowledge, this is the 19th case of JDM reported for generalized edema in the English literature. q

The presence of megacapillaries and a decreased capillary density are the hallmarks of the scleroderma capillary pattern, which can be detected by nailfold capillarmicroscopy. One hundred and eighty-six patients with Raynaud's phenomenon, 65 cases with undifferentiated connective tissue disease (UCTD), 47 patients with systemic lupus erythematosus (SLE), 26 patients with dermato/polymyositis, 14 with rheumatoid arthritis, seven cases with primary Sjögren's syndrome and 102 patients with systemic sclerosis (SSc) were investigated. Of the 16 patients with diffuse cutaneous SSc and the 86 limited cutaneous SSc cases, 14 (87.5%) and 53 (61.6%) showed the scleroderma capillary pattern, respectively. Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with Raynaud's phenomenon also exhibited the same pattern. Four of the 47 (8.5%) with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or Sjögren's syndrome, exhibited the scleroderma capillary pattern. The conclusion is that the scleroderma capillary pattern is often present in SSc and dermato/polymyositis. Furthermore, patients with Raynaud's phenomenon and UCTD may also occasionally exhibit this pattern. Therefore, capillarmicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing scleroderma spectrum disorders.

Dermatomyositis, a rare inflammatory disease possibly of autoimmune origin, produces a characteristic skin rash and symmetrical proximal myopathy. Patients with Dermatomyositis have a higher incidence rate of cancer, compared with the general
population, since DM usually presents as a paraneoplastic syndrome. through the study of 9 cases of paraneoplastic dermatomyositis we will discuss the clinical feature, paraclinical, etiological, therapeutic and outcomes of paraneoplastic dermatomyositis
(PDM) relative to the literature.

Ragged red fibers are an important marker for mitochondrial disease. To evaluate the hypothesis that mitochondrial dysfunction may play a role in the pathogenesis of aging and inclusion body myositis, we studied the frequency of ragged red fibers in muscle biopsy specimens from 15 young and 13 old normal adults, and from 27 patients with inclusion body myositis, polymyositis, or dermatomyositis. Serial transverse cryostat sections were stained with modified Gomori trichrome, modified succinic dehydrogenase, and cytochrome c oxidase. The frequency of ragged red fibers, determined by measuring the percent number of succinic dehydrogenase-positive ragged red fiber equivalents, was significantly higher in old compared to young normal subjects (0.33 vs. 0.02%, p < 0.0001). With the exception of a single polymyositis biopsy specimen showing a large number of ragged red fibers, the frequency of ragged red fibers in patients with polymyosii i or dermatomyositis was similar to that of age-matched normal control subjects. The frequency of ragged red fibeis was more than 1% in 7 of 8 patients with inclusion body myositis (maximum, 15%). The modified succinic dehydrogenase stain was more sensitive than the modified Gomori trichrome in detecting accumulation of mitochondria in muscle fibers. Cytochrome c oxidase activity was deficient in most ragged red fibers. We conclude that the number of ragged red fibers increases with normal aging and may reflect an age-related decline in muscle mitochondrial oxidative metabolism. The frequent occurrence of ragged red fibers in inclusion body myositis suggests that mitochondrial function may be impaired in this disease.

Dermatomyositis (DM) can occur in both adults and juveniles with considerable clinical differences. The links between immune-mediated mechanisms and vasculopathy with respect to development of perifascicular pathology are incompletely understood. We investigated skeletal muscle from newly diagnosed, treatment-naïve juvenile (jDM) and adult dermatomyositis (aDM) patients focusing on hypoxia-related pathomechanisms, vessel pathology, and immune mechanisms especially in the perifascicular region. Therefore, we assessed the skeletal muscle biopsies from 21 aDM, and 15 jDM patients by immunohistochemistry and electron microscopy. Transcriptional analyses of genes involved in hypoxia, as well as in innate and adaptive immunity were performed by quantitative Polymerase chain reaction (qPCR) of whole tissue cross sections including perifascicular muscle fibers.Through these analysis, we found that basic features of DM, like perifascicular atrophy and inflammatory infiltrates, were present a...

Péricardite révélatrice d'une dermatomyosite Pericarditis revealing a dermatomyositis La dermatomyosite (DM) fait partie des myopathies inflammatoires, dont le diagnostic positif repose sur des critères cliniques surtout musculaires et depuis une quinzaine d'années, sur la présence de certains auto-anticorps spécifiques de ces myosites [1]. L'atteinte cardiaque au cours des DM est fréquente, apparaissant essentiellement au cours de l'évolution de la maladie. Elle peut exceptionnellement être inaugurale. Nous rapportons une nouvelle observation de DM révélée par une péricardite. Observation Un patient âgé de 56 ans, sans antécédents pathologiques notables, a été hospitalisé pour une dyspnée d'effort évoluant depuis six mois, avec aggravation progressive, vers une dyspnée de repos stade III de NYHA depuis deux semaines. L'interrogatoire a révélé l'installation progressive depuis quatre mois, de myalgies de topographie rhizomélique, associées à une polyarthralgie inflammatoire touchant les grosses et les petites articulations (genoux et petites articulations des mains), avec détérioration de l'état général et fièvre à 38 8C. À l'examen clinique, le patient était dyspnéique et tachycarde (à 89 battements/min). Un oedème des membres inférieurs mous, blancs, gardant le godet a été noté, mais sans hépatomégalie, ni turgescence des jugulaires. La palpation du deltoïde et des muscles fessiers était douloureuse, associée à un déficit musculaire des ceintures. Une oligoarthrite des genoux a également été constatée. L'auscultation cardiaque a révélé un assourdissement des bruits du coeur avec une tachycardie sinusale à l'électrocardiogramme. Par ailleurs, à l'examen du tégument, on a trouvé un érythème oedémateux, rose violacé, des paupières « en lunette », s'étendant aux éminences malaires, associé à des papules de Gottron au niveau du dos des mains (figure 1A et B). Des plaques érythémato-squameuses ulcérées des coudes et des genoux ont été également observées. Un syndrome inflammatoire biologique a été noté avec une VS à 80 mm à la première heure et une C-reactive protein (CRP) à 67 mg/L. Les enzymes musculaires étaient de 8600 UI/L pour les créatine-phosphokinases (CPK) (valeurs normales : 0-145) et les lactates déshydrogénases (LDH) étaient de 1055 UI/L

Objective. To identify preliminary core sets of outcome variables for disease activity and damage assessment in juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM). Methods. Two questionnaire surveys were mailed to 267 physicians from 46 different countries asking each member to select and rank the response variables used when assessing clinical response in patients with JSLE or JDM. Next, 40 paediatric rheumatologists from 34 countries met and, using the nominal group technique, selected the domains to be included in the disease activity and damage core sets for JSLE and JDM. Results. A total of 41 response variables for JSLE and 37 response variables for JDM were selected and ranked through the questionnaire surveys. In the consensus conference, domains selected for both JSLE and JDM activity or damage core sets included the physician and parent/patient subjective assessments and a global score tool. Domains specific for JSLE activity were the immunological tests and the kidney function parameters. Concerning JDM,

such as cutaneous vasculitis (4,s) and rOSacea (5-7) give rise to epidermal basement membrane zone immunoglobulin deposition. The bands of immunoglobulin present in these disorders tend to he less intense and more irregular than the immunoglobulin band found in vised form December 26. IY79.

Heat-shockistress proteins (HSPs) are induced in response to stressful conditions and are essential for survival during and after cellular stress. We investigated whether dystrophin deficiency in muscle from Duchenne muscular dystrophy (DMD) patients induces HSPs. Immunohistochemical studies were performed on cryosections from normal muscle, heat-shocked muscle, and muscle from patients with DMD, dermatomyositis, and mitochondrial myopathy using antibodies against HSP 72/73, HSP 72, HSP 90, groEL (HSP 65 homologue), and ubiquitin. Computer-assisted image processing revealed a significant (P < 0.05) induction of HSP 72/73, 72, 65, and ubiquitin in hypercontracted fibers; HSP 90 and ubiquitin in regenerating fibers; and ubiquitin in macrophage invaded necrotic fibers of DMD muscle. No significant induction of HSPs was observed i n dermatomyositis or mitochondrial myopathies. The stress response induced in DMD may relate to the metabolic stress characteristic of the disease and could represent an autoprotective mechanism. Manipulation of this protective response may reduce injury and have potential therapeutic application. 0 1995 John Wiley & Sons, Inc.

Skin involvement in dermatomyositis does not always parallel muscle disease and can be the most active or severe component of the disease, failing to respond to therapeutic interventions that are adequate for myositis and other systemic involvement. This case refers to a resistant paraneoplastic amyopathic dermatomyositis rash which readily resolved with intravenous immunoglobulin (IVIG), practically in the form of monotherapy. This observation gains particular significance considering that IVIG is still regarded as a second-line therapeutic regimen in the treatment of dermatomyositis despite its prevalence in matters of safety.

Monozygotic (MZ) twins are partially concordant for most complex diseases, including autoimmune disorders. Whereas phenotypic concordance can be used to study heritability, discordance suggests the role of non-genetic factors. In autoimmune diseases, environmentally driven epigenetic changes are thought to contribute to their etiology. Here we report the first high-throughput and candidate sequence analyses of DNA methylation to investigate discordance for autoimmune disease in twins. We used a cohort of MZ twins discordant for three diseases whose clinical signs often overlap: systemic lupus erythematosus (SLE), rheumatoid arthritis, and dermatomyositis. Only MZ twins discordant for SLE featured widespread changes in the DNA methylation status of a significant number of genes. Gene ontology analysis revealed enrichment in categories associated with immune function. Individual analysis confirmed the existence of DNA methylation and expression changes in genes relevant to SLE pathogenesis. These changes occurred in parallel with a global decrease in the 5-methylcytosine content that was concomitantly accompanied with changes in DNA methylation and expression levels of ribosomal RNA genes, although no changes in repetitive sequences were found. Our findings not only identify potentially relevant DNA methylation markers for the clinical characterization of SLE patients but also support the notion that epigenetic changes may be critical in the clinical manifestations of autoimmune disease.

We report the case of a 46-year-old woman presenting cutaneous lesions similar to those of pityriasis rubra pilaris, characterized by diffuse scaling erythroderma and palmoplantar keratoderma; skin biopsy showed follicular hyperkeratosis with a perivascular lymphocytic infiltrate. One year later she developed an inflammatory myopathy; on the basis of clinical features, a diagnosis of dermatomyositis was made. Treatment with prednisone and hydroxychloroquine led to complete control of the cutaneous and muscular involvement, which was maintained during prednisone tapering.

Introduction: The aim of this study was to investigate the expression of tumor necrosis factor-like weak inducer of apoptosis (TWEAK) and its receptor fibroblast growth factor-inducible 14 (Fn14) in patients with polymyositis (PM) and dermatomyositis (DM), and their relation to clinical manifestations. Methods: Serum levels of TWEAK were detected in 98 PM/DM patients and 37 healthy controls by using the ELISA method. Total RNA isolated from fresh-frozen muscle tissue samples of 36 PM/DM patients and 10 healthy controls were used for analyzing the mRNA levels of TWEAK and Fn14 by quantitative reverse transcription polymerase chain reaction (RT-PCR). Immunofluorescence staining of TWEAK and Fn14 was conducted on muscle biopsy specimens from 23 PM/DM patients and seven healthy controls. Results: Serum levels of TWEAK were significantly decreased in the PM/DM patients compared to those in the healthy controls (P < 0.001), and serum TWEAK levels negatively correlated with serum CD163 levels in PM/DM patients (r =-0.49, P < 0.001). The expression of Fn14 mRNA was significantly increased in the muscle tissue of PM/ DM patients than in the muscle tissue of healthy controls (P < 0.01), whereas the expression of TWEAK mRNA in PM/DM patients was not statistically different from that of the healthy controls (P > 0.05). Fn14 mRNA levels in muscle tissue positively correlated with muscle disease activity (r = 0.512, P < 0.01). Patients with oropharyngeal dysphagia had significantly higher Fn14 mRNA levels than patients without oropharyngeal dysphagia (P < 0.05). The results of immunofluorescence staining showed that 19 out of 23 PM/DM patients were TWEAK-positive, and 20 out of 23 PM/DM patients were Fn14-positive. No detectable expressions of TWEAK or Fn14 were observed in the healthy controls. Conclusions: TWEAK-Fn14 axis may be involved in the pathogenesis of PM/DM. Further understanding of TWEAK-Fn14 function in PM/DM may help to define therapeutic targets for PM/DM.

Breast cancer is the leading cause of death from malignancy in women worldwide, with nearly 500,000 deaths each year. Its diagnosis must be a priority for world health. Breast cancer is the second tumor that can produce a paraneoplastic syndrome after lung cancer. Dermatomyositis with a diagnosis in later ages correlates significantly with an underlying malignancy. Therefore, you should always rule out the presence of a common disease (breast cancer) with the presentation of a rare disease (dermatomyositis).

Objective: The current study was performed in order to determine the prevalence of different myositisspecific and myositis-associated antibodies, as well as their association with clinical characteristics, disease course and response to therapy in 169 Hungarian patients with idiopathic inflammatory myopathy. Methods: Sera of 130 primary and 39 overlap myositis including systemic sclerosis (13), rheumatoid arthritis (12), systemic lupus erythematosus (5) and Sjögren's syndrome (9) cases were analyzed. Antinuclear antibody, scleroderma-associated antibodies (anti-centromere, anti-topoisomerase I), anti-Jo-1, anti-PL-7, anti-PL-12, anti-Mi-2, anti-SRP and anti-PM-Scl, anti-Ku, anti-SS-A, anti-SS-B, anti-U1snRNP were tested. Autoantibody results were compared with clinical characteristics, disease course of overlap versus primary myositis patients, as well as with response to therapy. Results: Associated connective tissue disease occurred in 23.1% of the patients. Myositis-associated antibodies were found in 8.5% of primary myositis patients, indicating that 11 additional primary myositis patients (23% vs. 29.6%) can be classified as overlap in all cohort according to the newly proposed diagnostic criteria. Polymyositis was found to be the most common myositis form in overlap myositis (87.2%), while scleroderma was the most common disease associated (33.3%). ANA was positive in 25.4% of primary and in 61.5% of overlap myositis cases. Altogether 39.6% of myositis patients (n = 67) had autoantibodies, most commonly anti Jo-1 (18.3%) correlating with a polycyclic disease course. Conclusion: Inclusion of myositis-specific and associated antibodies into the newly proposed diagnostic criteria for inflammatory myopathies is of great importance in order to determine subclasses and to introduce adequate therapy in time.

Background: The 12-lead surface electrocardiogram (ECG) is a useful tool to predict both atrial and ventricular arrhythmias via P-wave and QT measurements and its derivatives. Polycythemia vera (PV) is a chronic myeloproliferative disorder associated with cardiovascular events. The aim of this study was to assess ECG findings of patients with PV. Method and materials: Sixty patients with PV (34 male, mean age 58±11 years) and 60 age and gender-matched healthy volunteers were enrolled into the study. From the 12-lead surface ECG, P-wave and both conventional QT measurements and transmyocardial repolarization parameters (T peak -T end interval (T p -T e ) and derivatives) were evaluated digitally by two experienced cardiologists. In addition, a novel parameter, Pi was calculated digitally as the standard deviation of the P-wave duration across the 12 ECG leads. Results: QT duration and corrected QT interval were significantly longer in the PV group compared to healthy controls (p<0.01 and p<0.01, respectively). The T p -T e was longer and the T p -T e /QT ratio was significantly higher in the PV group compared to the controls. P-wave analyses showed that all P-wave parameters including Pmax, Pmin, P dispersion, and Pi were significantly prolonged in PV patients compared to the controls. The increase of both T p -T e and P max in the PV group was independent of age, BMI, diabetes and hypertension, gender, systolic blood pressure, hemoglobin, hematocrit, left atrial dimension, left ventricular end-diastolic diameter and early deceleration time in a univariate analysis of co-variance model (F=11.097, p=0.001 and F=31.537, p=0.0001, respectively).

ObjectiveTo validate and promulgate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile systemic lupus erythematosus (SLE).To validate and promulgate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile systemic lupus erythematosus (SLE).MethodsIn 2001, a preliminary consensus-derived core set of measures for evaluating the response to therapy in juvenile SLE was established. In the present study, the core set was validated through an evidence-based, large-scale data collection process that led to the enrollment of 557 patients from 39 different countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, agreement in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response.In 2001, a preliminary consensus-derived core set of measures for evaluating the response to therapy in juvenile SLE was established. In the present study, the core set was validated through an evidence-based, large-scale data collection process that led to the enrollment of 557 patients from 39 different countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, agreement in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response.ResultsThe following clinical measures were found to be feasible and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) global disease activity measure, 3) 24-hour proteinuria, 4) parent's global assessment of the patient's overall well-being, and 5) health-related quality of life assessment.The following clinical measures were found to be feasible and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) global disease activity measure, 3) 24-hour proteinuria, 4) parent's global assessment of the patient's overall well-being, and 5) health-related quality of life assessment.ConclusionThe members of PRINTO propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a patient with juvenile SLE has responded adequately to therapy.The members of PRINTO propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a patient with juvenile SLE has responded adequately to therapy.

To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. Design: Retrospective study. Setting: University hospitals. Patients: Thirteen patients with amyopathic dermatomyositis. Results: The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. Conclusions: Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.