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At least 883 records · Page 49Linked to original sources
PubMed1976

Dermatomyositis in childhood. Review of eight cases.

Eight cases of dermatomyositis in children admitted to Scottish hospitals between 1962 and 1972 have been reviewed. 6 of the 8 were currently in complete remission. In the other 2 cases the disease remained active in 1 and 1 had died of cardiac failure 6 years after the onset of disease. 5 had developed extensive soft tissue calcification for which 2 were treated with ethanehydroxydiphosphonate, one showing definite improvement and the other no change. All had been treated with corticosteroids and two in addition had had cytotoxic agents (methotrexate or cyclophosphamide). The overal prognosis had probably been improved by the use of corticosteroids but not by the cytotoxic drugs. Only one of the patients was incapacitated by residual contractures or calcinosis.

Biopsy↗
PubMed1983

Drug treatment of juvenile dermatomyositis.

A series of 29 children with dermatomyositis has been reviewed and the outcome compared between cases treated by us initially with a low dosage short duration course of corticosteroids, and those referred late and having had various different therapeutic regimens and usually more active and higher dosage drug schedules. There were fewer relapses and less morbidity in the low dosage short term group. It was noted also that there was no relation between the initial mode of onset or severity and the eventual outcome or course of the disease. However, pronounced skin vasculitis appeared to reflect severity of disease.

Acute Disease↗
PubMed2006

Signs of inflammation in both symptomatic and asymptomatic muscles from patients with polymyositis and dermatomyositis.

OBJECTIVES: To determine whether muscle weakness is correlated with inflammation, expression of interleukin 1alpha (IL1alpha) and major histocompatibility complex (MHC) class I and II antigens on muscle fibres. METHODS: Biopsy specimens from clinically symptomatic (proximal muscles) and asymptomatic (all distal but two proximal) muscles in eight patients with polymyositis, three patients with dermatomyositis and six healthy controls were analysed by immunohistochemistry for the presence of T cells and macrophages, and expression of IL1alpha and of MHC class I and II antigens. RESULTS: were evaluated by conventional light microscopy and by computerised image analysis. Results: Inflammatory infiltrates with T cells and macrophages were observed to an equal degree in both symptomatic and asymptomatic muscle. The numbers of capillaries with IL1alpha expression were significantly higher (p<0.05) in the symptomatic and asymptomatic muscles of patients than in controls. The total IL1alpha expression per tissue section assessed by computerised image analysis was significantly higher in symptomatic muscles but not in asymptomatic muscles compared with that in controls. Neither the number of IL1alpha-positive capillaries nor the total IL1alpha expression differed significantly between symptomatic and asymptomatic muscles. Expression of MHC class I and II antigens on muscle fibres was detected in both symptomatic and asymptomatic muscles but rarely in healthy controls. CONCLUSIONS: Presence of inflammatory infiltrates, T cells and macrophages, and expression of MHC class I and II antigens and of IL1alpha on muscle fibres were independent of clinical symptoms, and were present to an equal degree in both proximal and distal muscles. Thus, other factors seem to determine the development of clinical symptoms. One such factor could be variations in physical demands.

Adult↗
PubMed1977

Dermatomyositis complicating penicillamine treatment.

A case of dermatomyositis developing during the course of treatment with D-penicillamine in a patient with rheumatoid arthritis is described. Complete remission occurred on withdrawal of the drug. Possible alternative diagnoses are discussed.

Arthritis, Rheumatoid↗
PubMed1980

Muscle biopsy in polymyositis and dermatomyositis: a clinicopathological study.

The initial muscle biopsies of 30 patients with active polymyositis or dermatomyositis have been assessed histologically and morphometrically. Only 1 patient (3.3%) had a normal biopsy, while 3 had a normal electromyograph and 8 had a normal serum CPK level. No significant morphological differences were found between the various diagnostic subgroups. Sequential biopsies of 11 of these patients treated either with corticosteroids only or with intensive immunosuppression were studied. With response to treatment atrophy factors, variability coefficients, internal nuclei count, and the various histological changes improved in the whole group of patients. However, there was no consistent correlation between any of the morphological features or between these and clinical parameters in individual patients. The percentage of fibres with internal nuclei in the initial biopsy was lower (P < 0.001) in patients responding to up to 60 mg prednisolone per day than in those who eventually required cytotoxic drugs in addition.

Adolescent↗
PubMed1987

Preliminary observations on the role of magnetic resonance imaging for polymyositis and dermatomyositis.

The potential use of magnetic resonance imaging (MRI) for inflammatory muscle disorders was evaluated in 13 patients with polymyositis and dermatomyositis. Abnormalities in signal intensity (p = 0.0076) and fat replacement (p = 0.0177) were identified and correlated significantly with clinical disease activity. In addition, MRI was useful in directing muscle biopsy of selected abnormal areas.

Adolescent↗
PubMed1988

Constrictive pericarditis complicating dermatomyositis.

Chronic constrictive pericarditis may complicate both rheumatoid arthritis and systemic lupus erythematosus. To our knowledge, however, this is the first time it has been described in a patient with dermatomyositis. This association should be kept in mind as constrictive pericarditis should be considered in the differential diagnosis of liver cirrhosis and Budd-Chiari syndrome in rheumatic diseases.

Dermatomyositis↗
PubMed2006

Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis.

Progressive multifocal leucoencephalopathy (PML), a demyelinating disease caused by the JC virus (JCV), occurs in immunosuppressed patients and carries a poor prognosis. A favourable outcome is reported in two patients with PML and dermatomyositis. Immunosuppressive drugs were stopped in patient 1 but could only be partially tapered in patient 2. The JCV-specific CD8+ T cell response was strong in patient 1 and weak in patient 2. Both were treated with cytosine-arabinoside, and patient 2 was also treated with mirtazapine, a 5HT2A receptor antagonist. Combination of these drugs might be helpful to treat HIV-negative patients with PML.

Adrenergic alpha-Antagonists↗
PubMed1975

Mitogen and muscle extract induced in vitro proliferative responses in myasthenia gravis, dermatomyositis, and polymyositis.

We have compared the lymphocyte in vitro proliferative response induced by muscle extract, phytohaemagglutinin, and pokeweed mitogen in peripheral blood lymphocytes of patients with myasthenia gravis, polymyositis, dermatomyositis, and normal subjects. Similar mean responses to antigen and mitogen were seen in cells of all groups of patients. The proliferative responses induced by muscle did not differ in thymic cells from myasthenics as compared with those from cardiac surgery controls. Our findings do not support the hypothesis that cell-mediated immunity to muscle or major impairment of lymphocyte responses to mitogens occurs in these diseases.

Dermatomyositis↗
PubMed1976

Serum immunogloblin levels in myasthenia gravis, polymyositis, and dermatomyositis.

Analyses of serum immunoglobulin (Ig) levels were performed in patients with myasthenia gravis, polymyositis and dermatomyositis, and in normal subjects. The mean IgG levels were elevated in the disease groups and there was minimal depression of serum IgM levels in nonthymectomized myasthenic patients. There was no consistent pattern with regard to selective immunoglobulin class abnormalities, effect of thymectomy in serial studies, and no evidence of gross humoral immunological deficiency as manifested by consistent depression of serum IgA, IgG, or IgM in the disease groups.

Adult↗
PubMed1991

Cyclosporin in the management of polymyositis and dermatomyositis.

Three patients with polymyositis refractory to conventional steroid and immunosuppressive treatment, but responsive to cyclosporin A, are described. In a fourth patient cyclosporin A was used as a first line drug in combination with steroids in the treatment of life-threatening dermatomyositis. Cyclosporin A in the management of polymyositis/dermatomyositis requires formal assessment of its costs and benefits compared with conventional treatments.

Adult↗
PubMed1990

Dermatomyositis: correlative MR imaging and P-31 MR spectroscopy for quantitative characterization of inflammatory disease.

Magnetic resonance (MR) imaging and phosphorus-31 MR spectroscopy were used to examine four patients with dermatomyositis and five control subjects. T2-weighted images of the thigh muscles of patients showed increased signal intensity, with focal and inhomogeneous involvement predominantly in the vastus lateralis and secondarily in the vastus intermedius and vastus medialis. T1 and T2 values of the vastus lateralis in patients were significantly higher than those of the control subjects. T1 values of the rectus femoris and biceps femoris with more generalized inflammation were moderately elevated but still significantly higher than those of the control subjects. P-31 MR spectra of the quadriceps muscles were obtained during rest, during exercise at two graded levels, and in recovery. Concentrations of adenosine triphosphate and phosphocreatine (PCr) in the diseased muscles were 30% below normal values, and the inorganic phosphate/PCr ratios were increased in the patients' muscles at rest and throughout exercise. The T1 and T2 values as well as the P-31 metabolite data correlated with symptoms and clinical assessment.

Adult↗
PubMed2005

[Juvenile amyopathic dermatomyositis and calcinosis].

Juvenile dermatomyositis (JDM) is a chronic multisystemic disease. It is believed to be of autoimmune etiology and is characterized by the presence of vasculitis affecting striated muscle and skin. Clinical description consists of general symptoms (anorexia, weight loss, asthenia, fever), typical manifestations (muscular and cutaneous) and possible systemic alterations. We report the case of a 5-year-old boy diagnosed with JDM who presented difficulty in walking, inability to completely extend the right lower extremity and calcinosis 12 months after the development of incorrectly evaluated cutaneous alterations. We aim to highlight the importance of early diagnosis and treatment of this illness. Since the introduction of corticosteroid therapy, prognosis has improved but functional outcome still depends on the presence of calcinosis and muscular contractures.

Calcinosis↗
PubMed2001

Cutaneous leukocytoclastic vasculitis in dermatomyositis suggests malignancy.

BACKGROUND: Dermatomyositis (DM) is a rare connective tissue disease which has been shown to be associated with an underlying malignancy. OBJECTIVE: Evaluation of the prevalence of malignancy in DM at our clinic and search for characteristics of the paraneoplastic form of disease. METHODS: Retrospective review of patient files and histology reports over the period from 1991 to 1998. RESULTS: 23 patients (14 women and 9 men) with DM could be identified in this time period with a median age at diagnosis of 48 years. Malignancies were found in 5 (22%) cases. The skin biopsies of all patients showed features of DM; in 7 cases, a leukocytoclastic vasculitis was detected. Four of the 5 cases with an associated malignancy demonstrated histologically a vasculitis in lesional skin, compared to only 3 out of 18 cases without malignancy (p < 0.05, Fisher's exact test). CONCLUSION: Our data suggest that vasculitis in lesional skin biopsies has a predictive value for the presence of underlying malignancy.

Adult↗
PubMed1977

Methotrexate treatment in dermatomyositis.

Two patients with dermatomyositis were treated with methotrexate, given intravenously. They were unresponsive to corticosteroids. The patients were bedridden and paralysed and one seemed to be in the terminal phase of the disease. The response to courses of methotrexate treatment was satisfactory and sustained over a 2-year follow-up period; muscle enzyme studies indicate that the muscle disease is no longer active. Although steroid therapy alone may be effective, its usefulness is unpredictable and may lead to dependence and undesirable side effects. Our experience with these two patients suggests that a combination of the two drugs may be justified as initial treatment in severe cases.

Aspartate Aminotransferases↗