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PubMed2003

The physiological and physical determinants of functional ability measures in children with juvenile dermatomyositis.

OBJECTIVE: To study the relationships of muscle strength and maximal oxygen consumption (VO(2peak)) with Childhood Health Assessment Questionnaire (CHAQ) score, Childhood Myositis Assessment Score (CMAS) and Child Health Questionnaire [physical summary (CHQ-PhS) and psychosocial summary (CHQ-PsS)] scores in juvenile dermatomyositis. METHOD: Fifteen patients (age 5-14 yr) participated. CMAS, CHAQ, CHQ, muscle strength and VO(2peak) were measured. RESULTS: Correlations revealed significant relationships between CHAQ and (i) muscle strength (r=-0.72) and (ii) absolute VO(2peak) (r=-0.68); between CMAS and relative VO(2peak) (r=0.73); and between CHQ-PhS and (i) muscle strength (r=0.57) and (ii) relative VO(2peak) (r=0.58). Backward regression analysis showed that muscle strength was the best indicator of variation in CHAQ. Age and relative VO(2peak) were the best indicators for CMAS. Body mass and age were the best indicators for CHQ-PsS. Body mass and muscle strength were the best indicators for CHQ-PhS. CONCLUSION: CMAS, CHAQ and CHQ correlate with muscle strength and VO(2peak). CMAS, CHAQ and CHQ depend on different physical and physiological variables.

Activities of Daily Living↗
PubMed2003

Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis.

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, functional ability and muscle strength assessments were indicated for both activity and damage core sets, whereas serum muscle enzymes were included only in the activity core set. A specific paediatric domain called 'growth and development' was introduced in the disease damage core set for both diseases and the evaluation of health-related quality of life was advised in order to capture the influence of the disease on the patient lifestyle. CONCLUSIONS: We developed preliminary core sets of measures for disease activity and damage assessment in JSLE and JDM. The prospective validation of the core sets is in progress.

Child↗
PubMed2004

Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety.

OBJECTIVES: To assess the efficacy and safety of intravenous cyclophosphamide (CYP) used in severe and refractory juvenile dermatomyositis (JDM). METHODS: Retrospective case note review of the outcome of 12 patients. RESULTS: Assessment at 6 months of therapy in 10 of the 12 patients showed a significant improvement in muscle function as assessed by the Childhood Myositis Assessment Scale (CMAS) (P = 0.012), muscle strength (P = 0.008), global extramuscular disease score (P = 0.008), skin disease severity (P = 0.015) and lactate dehydrogenase (P = 0.028). There were reductions in creatine kinase, alanine aminotransferase, prednisolone dose and ESR, but these did not reach statistical significance. Clinical improvement was maintained after CYP until the most recent follow-up (between 6 months and 7 yr) and no severe side-effects were seen. Reversible complications included lymphopenia, herpes zoster infections and alopecia. The median cumulative dose was 4.6 g/m(2) (range 3-9 g/m(2)). The available evidence suggests that, at the doses required, risks of malignancy, infertility and gonadal failure are low. Two patients with severe treatment-resistant disease died after one dose of CYP, both of whom were ventilated prior to commencement of CYP and were thought to have died as a result of their severe disease process, and too early for clinical benefit to be obtained from the drug. CONCLUSIONS: In this cohort of children with severe and refractory JDM, CYP appeared to have provided major clinical benefit with no evidence of serious toxicity in the short term.

Antirheumatic Agents↗
PubMed2004

Quantitative assessment of MRI T2 relaxation time of thigh muscles in juvenile dermatomyositis.

OBJECTIVE: The aim of the study was to examine the validity and reliability of a quantifiable measure of inflammation using magnetic resonance imaging (MRI) in children with juvenile dermatomyositis (JDM). METHODS: Children with active JDM, inactive JDM and healthy children received detailed assessments of recognized measures of muscle inflammation including muscle strength (manual muscle testing and myometry) and function (Childhood Myositis Assessment Scale, Childhood Health Assessment Questionnaire), the muscle enzymes lactate dehydrogenase (LDH) and creatine kinase (CK) and T2-weighted MRI scans of the thigh muscles, and these values were correlated with each other. RESULTS: Ten children with active JDM, 10 with inactive JDM and 20 healthy children completed the study. There was no significant difference in ages between the three groups. The MRI T2 relaxation times were significantly increased in active JDM compared with inactive JDM and healthy children (P = 0.05), indicating a detectable increase in inflammation within the muscles. There were also good correlations between the MRI scores and the measures of muscle strength and function; however, there was no correlation between the MRI and muscle enzymes. CONCLUSIONS: The MRI T2 relaxation time can be used as a quantitative measure of muscle inflammation and it has good correlations with other measures of disease activity.

Adolescent↗
PubMed2004

Metabolic disturbances during short exercises in dermatomyositis revealed by real-time functional 31P magnetic resonance spectroscopy.

OBJECTIVE: (31)P magnetic resonance spectroscopy (MRS) is useful for evaluating metabolic disturbances in dermatomyositis (DM). However, short-term alterations of metabolic parameters such as Pi/PCr (inorganic phosphate/phosphocreatine) have not been assessed in detail, although they may reveal insights into the origin of the known long-term changes. We therefore performed real-time functional (31)P MRS to find out if there are characteristic short-term alterations of metabolic dynamics during muscular exercise and if they are of diagnostic relevance. METHODS: MRS measurements were performed on lower calf muscles of 10 DM patients and 18 healthy subjects throughout five short (1 min) cycles of submaximal exercise (50% maximum voluntary contraction). RESULTS: Pi/PCr ratios during exercise increased in patients and controls. They rapidly returned to baseline values in the controls, but both Pi and PCr remained above baseline values in patients and resulted in irregular Pi/PCr ratios. This was true for each individual patient, but resulted in broad variation in individual Pi/PCr values. To compare groups with limited patient numbers, it was therefore more appropriate to use a recovery index, i.e. the quotient of the Pi/PCr ratio during and after exercise, which was independent of individual parameters, such as age and the work/energy cost ratio. CONCLUSION: Evaluation of short-term changes by real-time functional (31)P MRS provides insight into alterations of Pi/PCr ratios and could improve diagnostic parameters in DM.

Adenosine Triphosphate↗
PubMed2006

Identification of a novel autoantibody reactive with 155 and 140 kDa nuclear proteins in patients with dermatomyositis: an association with malignancy.

OBJECTIVE: Myositis-specific autoantibodies (MSAs) are a useful tool in diagnosis, defining clinical subsets and predicting prognosis of dermatomyositis (DM) and polymyositis (PM). In this study, we identified a novel MSA reactive with 155 and 140 kDa nuclear proteins [anti-155/140 antibody (Ab)] and determined the clinical feature of DM patients positive for this autoantibody (autoAb). METHODS: Sera from 52 Japanese patients with DM, 9 with PM, 48 with systemic lupus erythematosus (SLE), 126 with systemic sclerosis and 18 with idiopathic interstitial pneumonia were examined by immunoprecipitation assays. Positive sera were further characterized by immunodepletion and immunofluorescence staining. RESULTS: Seven of the 52 (13%) Japanese patients with DM immunoprecipitated 155 and 140 kDa proteins from 35S-labelled K562 leukaemia cell extract. No patients with SLE, systemic sclerosis or idiopathic interstitial pneumonia as well as healthy controls were positive for this autoAb. Patients with anti-155/140 Ab developed heliotrope rash, Gottron's papules or sign and flagellate erythema significantly more frequently than those negative. Notably, internal malignancy was found at significantly higher frequency in those positive than those negative (71 vs 11%; P < 0.005). In contrast, none of these patients positive for this autoAb had interstitial lung disease. CONCLUSIONS: This novel MSA is associated with cancer-associated DM and may serve as a diagnostic serological marker for this specific subset.

Adult↗
PubMed2006

The heart in dermatomyositis and polymyositis.

Cardiovascular manifestations constitute a major cause of death in myositis. Despite this, clinically manifest cardiac involvement in polymyositis and dermatomyositis is relatively rare. In contrast, subclinical manifestations are frequently reported and are predominated by conduction abnormalities and arrhythmias detected by ECG. The most frequently reported clinically overt manifestations are congestive heart failure, conduction abnormalities, that may lead to complete heart block, and coronary artery disease. The underlying pathophysiological mechanisms that may cause cardiac manifestations involve myocarditis and coronary artery disease as well as involvement of the small vessels of the myocardium.

Cause of Death↗
PubMed1996

Study of 45 cases of nasopharyngeal carcinoma with dermatomyositis.

The authors have conducted a study of 61 cases of nasopharyngeal carcinoma (NPC) with dermatomyositis (DM) admitted to the hospital between April 1964 and May 1989 and accounting for 0.027% (61/226, 183) of all the malignant tumors at the hospital and 0.086% (61/70,899) of the nasopharyngeal carcinoma cases, during that time period. We have analyzed 45 cases with complete data, using equal number of age-, sex-, and stage-matched cases with only NPC as control. The findings show a 5- and 10-year survival rate and distant metastatic rate of 50.4, 34.5, and 40.5% respectively, for NPC with DM, and 57.8, 55.2, and 56.5% for controls. The results indicated that the radiotherapy with prednisone treatment not only is quite effective but also will not result in a significantly increased rate of distant metastasis.

Adult↗
PubMed2004

High-grade neuroendocrine carcinoma of the lung presenting an unusual spread mimicking pleural mesothelioma associated with dermatomyositis.

Neuroendocrine tumors of the lung comprise a heterogeneous group of tumors that represents a spectrum of disease from typical carcinoid tumors to the high-grade neuroendocrine carcinomas (large-cell neuroendocrine carcinomas and small-cell carcinomas). The high-grade neuroendocrine carcinomas are characterized by early metastasis and poor prognosis. The peripheral location and especially the massive pleural spread are rare for a high-grade neuroendocrine carcinoma. We report a case in which a high-grade neuroendocrine carcinoma, associated with dermatomyositis, presented an unusual pattern of progression, mimicking malignant pleural mesothelioma on diagnostic imaging.

Carcinoma, Neuroendocrine↗
PubMed1980

Surgical treatment of subcutaneous calcifications of polymyositis or dermatomyositis.

The "tumoral" calcifications seen with polymyositis and dermatomyositis are frequently large and painful. Many surgeons believe such calcifications should not be removed surgically, arguing that the surgical trauma induces further calcification that is probably ever more severe. However, we believe that surgical removal of these large masses is less traumatic than cyclical, spontaneous extrusion of the calcium with secondary healing. We present 2 patients in whom chronically draining and painful dystrophic calcifications were treated surgically without recurrence.

Buttocks↗
PubMed1997

General and local scleroderma in children and dermatomyositis and associated syndromes.

Scleroderma in children virtually always manifests in the form of localized disease, whereas adult scleroderma usually manifests as systemic disease. The recent literature on childhood scleroderma is limited almost exclusively to localized disease. The treatment of this disease remains a dilemma, especially with regard to which patients should be treated, how they should be treated, and how the treatment response should be monitored. Our current therapeutic approach is reviewed. The recent literature regarding juvenile dermatomyositis is discussed. New understanding of the pathobiology of this disease should lead to improved, less toxic treatments in the future.

Child↗
PubMed2000

Current approach to the treatment of polymyositis and dermatomyositis.

Although corticosteroids remain the mainstay of treatment for the inflammatory myopathies, their use is complicated by many side effects. Other immunosuppressive agents, alone or in combination, are being increasingly used for patients with other severe disease or treatment-related complications. Pulmonary disease remains a serious source of morbidity and mortality in myositis patients. Cyclophosphamide, cyclosporine, and tacrolimus are efficacious in patients with interstitial lung disease. Intravenous immunoglobulin is not only effective for the cutaneous complications of dermatomyositis but has been helpful in other extramuscular manifestations.

Adrenal Cortex Hormones↗
PubMed2000

Interstitial lung disease in polymyositis and dermatomyositis.

Interstitial lung disease (ILD) is common in patients with polymyositis (PM) and dermatomyositis (DM), and is a major cause of morbidity. Although its cause is unknown, it is known to be closely associated with autoimmune disorders. Its manifestation has been found to be quite heterogeneous, as demonstrated by the differences among PM/DM patients in their immunologic profiles and histopathologic findings, which suggest variations in immunopathogenetic mechanisms. We review the clinicopathologic and immunologic findings in ILD associated with PM/DM, and discuss recent advances in classification, autoantibodies, and treatment. The most critical issues are to clarify the immunopathogenesis of severe forms of ILD, such as rapidly progressive ILD associated with amyopathic DM, and to establish the most appropriate therapy.

Dermatomyositis↗
PubMed2002

Clinical outcomes in juvenile dermatomyositis.

Juvenile dermatomyositis is a chronic inflammatory illness of unknown etiology that affects primarily muscle and skin. It has an incidence of 2-3 per 1,000,000, per year. The disease can affect other organ systems, including the gastrointestinal tract, lungs, and heart. In addition, calcinosis is seen in one-third of patients. The mainstay of therapy is corticosteroids; some children require additional immunosuppressive agents because of corticosteroid resistance or intolerance. Functional outcomes have become good with modern treatments, but the disease remains chronic in a large number of children and sequelae are often seen.

Calcinosis↗
PubMed1994

Dermatomyositis associated with Crohn's disease.

A 29-year-old woman with quiescent Crohn's disease developed severe myositis. Serum creatine phosphokinase was markedly elevated, and muscle biopsy showed typical findings of dermatomyositis. The muscular symptoms promptly responded to combined treatment with corticosteroids and azathioprine. The association between inflammatory bowel disease and inflammatory myopathies is very rare. We review the clinical, pathological, and pathophysiological aspects of this association.

Adult↗
PubMed1996

MHC class I-mediated cytotoxicity does not induce apoptosis in muscle fibers nor in inflammatory T cells: studies in patients with polymyositis, dermatomyositis, and inclusion body myositis.

Apoptosis plays a crucial role in natural recovery from T cell-mediated autoimmune disorders of the nervous system. Whether apoptosis also occurs in human inflammatory myopathies is unclear. In this study we examined muscle biopsy specimens from untreated patients with polymyositis (n = 12), dermatomyositis (n = 12), and inclusion body myositis (n = 12) for the presence of apoptosis using morphological criteria and DNA fragmentation by in situ tailing. In all these disorders, only rare T cells exhibited signs of apoptosis by nuclear morphology and in situ labeling techniques. Although Fas-expression was upregulated in a few inflammatory cells, increased apoptosis of the surrounding T cells was not observed. Further, nuclei of degenerating muscle fibers did not show morphological signs of apoptosis and were not labeled by the tailing reaction. We conclude that in the inflammatory myopathies, T cell inflammation is not cleared by apoptosis and affected muscle fibers do not die by apoptosis. The observations are consistent with the non-self-limited nature of these disorders and suggest that, in contrast to the nervous system, the local microenvironment in muscle does not deliver pro-apoptotic stimuli.

Adult↗
PubMed1997

Transforming growth factor-beta 1 in polymyositis and dermatomyositis correlates with fibrosis but not with mononuclear cell infiltrate.

The idiopathic inflammatory myopathies are diseases of unknown etiology characterized by T cell-mediated myocytotoxicity in polymyositis and complement-mediated angiopathy of muscle fibers in dermatomyositis. A variable degree of fibrosis is present in muscles in these conditions both perimysially and endomysially. We evaluated the expression of TGF-beta 1, a pleiotropic cytokine with fibrogenic and immunomodulating activity, by means of quantitative-polymerase chain reaction and immunocytochemistry in DM and PM muscle biopsies. TGF-beta 1 mRNA was significantly higher in DM compared with controls, whereas in PM the values were not significantly different when compared with controls and DM. TGF-beta 1 was localized in connective tissue but did not correspond with mononuclear cell infiltrates. These findings suggest a correlation between TGF-beta 1 and connective tissue proliferation in inflammatory myopathy, while its immunomodulatory role remains to be elucidated.

Adolescent↗
PubMed1990

Treatment of duodenocutaneous fistula with somatostatin analog in a child with dermatomyositis.

A 6-year-old girl with juvenile dermatomyositis complicated by five duodenal perforations is described. The treatment consisted of suture closure of the perforations, reinforcement of the suture line with an omental graft, and adequate drainage of the closure site. The use of a somatostatin analog was successful in promptly arresting a leak from the duodenal perforations that had persisted for 3 months after the operation despite prolonged bowel rest and total parenteral nutrition. On follow-up observation 2 months later, the patient had no abdominal complaints and was tolerating oral intake without problems.

Child↗