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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In contrast to the 15-20% incidence of the coexistence of acute dermatomyositis-polymyositis and malignancy, it has been accepted traditionally that the association of progressive systemic sclerosis, a disease with several features that may overlap the former condition, and malignancy is purely fortuitous. This experience has not been altered by the material presented in this review. However, the factual coexistence has been illuminated by a review of the pertinent literature and presentation of 12 previously unpublished case reports. Four cases concern pulmonary malignancies in PSS; eight are of an associated non-pulmonary malignancy. In the first group, the development of a malignancy superimposed on the chronic fibrosing changes in the lungs of PSS does not seem so strange, particularly in view of a possible immunologic reaction by collagen in considering pathogenesis. This immunologic process might be similar to a related immunologic process responsible for the development of malignant cells in pulmonary and other tissue, where normal cells usually are found. The high incidence of males is related to the high incidence of males in Veterans Administration Hospitals, the principal population source of these cases.
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PMID:Progressive systemic sclerosis (PSS) and malignancy, pulmonary and non-pulmonary. 21 19

Serum immunoreactive prolyl hydroxylase protein and galactosylhydroxylysyl glucosyltransferase activity were measured in 54 patients wtih various dermatological diseases and compared with corresponding values in 32 control subjects. These serum enzymes were at the control level in the great majority of the patients, and no correlation was found between serum and skin enzymes, except for one weak correlation in lichen ruber planus. Some patients with psoriasis, lichen ruber planus, keloids, erythema nodosum or chronic discoid lupus erythematosus, however, did have at least one of these enzymes elevated in the serum, and a significant correlation (P less than 0.01) between the two enzymes was found in the total disease material. Thus it does seem that diseases limited only to the skin can sometimes raise these serum enzyme levels. The mean levels of serum immunoreactive prolyl hydroxylase and galactosylhydroxylysyl glucosyltransferase activity were significantly elevated (P less than 0.001) in active systemic connective tissue diseases such as systemic lupus erythematosus, scleroderma or dermatomyositis compared with the controls, and 4 out of 7 values for immunoreactive prolyl hydroxylase and 3 out of 7 for galactosylhydroxylysyl glucosyltransferase activity were above the 95% confidence limit of the controls. Since the levels of the skin enzymes were not elevated in most of these patients, however, the main sources for the elevated serum enzymes were probably tissues other than the skin.
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PMID:Enzymes of collagen biosynthesis in skin and serum and dermatological diseases. II. Serum enzymes. 22 63

Muscle biopsies from 12 patients with a typical clinical picture of dermatomyositis have been examined by electron microscopy. Endothelial cells of intramuscular blood vessels, their basal lamina, pericytes, muscle fibers, and satellite cells show degenerative or regenerative alterations. In nine patients, tubular arrays were noted in the cisterns of endoplasmic reticulum of endothelial cells, pericytes, lymphocytes, macrophages and satellite cells. Other types of inclusions were also observed. The pathogenesis of the disease is discussed.
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PMID:An electron microscopic study of childhood dermatomyositis. 22 19

Muscle biopsies from 32 cases with polymyositis and dermatomyositis were examined by electron microscopy. Most of the changes of the muscle fibres were non-specific and little structural difference was seen in the various clinical types. The muscle fibres sometimes showed rough endoplasmic reticulum, annulate lamellae and prominent Golgi apparatus. Thin and thick filamentous inclusions were present both in the sarcoplasm and nuclei of some muscle fibres. Thick filamentous inclusions were seen in chronic cases and were sometimes associated with annulate lamellae. Regeneration of muscle was often conspicuous. The endothelial cells of the blood vessels were hypertrophied; some cells contained granulotubular inclusions, rod-shaped bodies (Weibel-Palade bodies) and filamentous material. The basement membrane of the vessels was often multi-layered. The cells infiltrating the interstitial tissue included macrophages, lymphocytes, transformed lymphocytes, plasma cells, monocytes, mast cells and only occasional eosinophils and basophils. Lymphocytes, macrophages and a few plasma cells were seen between the plasma and basement membranes of degenerating muscle fibres. Changes were also noted in some intramuscular nerves, motor end-plates and a muscle spindle.
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PMID:Ultrastructural changes in polymyositis. 22 91

The authors describe the case of a pulmonary interstitial fibrosis, the discovery of which preceeded the skin and muscular manifestations of dermatomyositis which was confirmed by a muscular biopsy. The aetiopathogenesis of the pulmonary disorder is not well known, nevertheless an immunological mechanism demonstrated in the experimental myositis is suggested but has yet to be proved.
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PMID:[Pulmonary fibrosis during a case of dermatomyositis (author's transl)]. 23 21

Two cases are presented which have been treated anticonvulsively for many years - especially with Phenytoin and Mephenytoin. The clinical syndrom as well as the changes of the connective tissue show transitions of Progressive systemic sklerosis (PSS) to systemic Lupus Erythematodes (SLE) and Dermatomyositis. The correlation with the therapy is reflected in consideration of immunological phenomenons.
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PMID:[A progressive systemic sclerosis like disease due to anticonvulsive therapy? (author's transl)]. 24 60

Forty-one children with dermatomyositis who were treated with corticosteroids and who have been followed over a period of 15 years have been presented. Progressive proximal muscle weakness was seen in all and 60% had muscle pain. The skin rash considered classic for dermatomyositis was seen in 33 children at the time of diagnosis. Elevation of serum muscle enzymes, electromyographic abnormalities, and muscle biopsy evidence of acute myositis were of confirmatory diagnostic value. The course of the disease in this study group has reconfirmed the efficacy of adrenal corticosteroid treatment in conjunction with an individualized physical therapy program and consistent followup. Prognosis for life and minimal functional disability has been good. There have been 3 deaths recorded in this series, only one of which was certain in its relationship to dermatomyositis.
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PMID:Dermatomyositis in the pediatric patient. 26 8

In this case of dermatomyositis, the patient's clinical course was complicated by the development of acute granulocytic leukemia. Various malignancies complicating dermatomyositis have been reported in the literature. Rarely have these been of the hematopoietic system.
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PMID:Dermatomyositis complicated by acute granulocytic leukeumia. 27 27

Modern treatment of childhood dermatomyositis with corticosteroids has resulted in greatly improved prognosis and style of life. The immunosuppressive drugs methotrexate and azathioprine have been utilized as ancillary agents in life-threatening disease and in children whose disease could not be adequately controlled with prednisone alone. Two patients are completely well without medication two and seven years after onset; two have received no medications for more than two years and have only subtle signs of any residual illness although they had been profoundly ill for two to three years after the onset of dermatomyositis. A fifth patient, completing the third year of disease, remains ill and continues to require medications but has improved sufficiently to return to regular class in school.
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PMID:Methotrexate and azathioprine treatment of childhood dermatomyositis. 29 38

Sera from 378 patients were assayed for antibodies to extractable nuclear antigens (ENA), ribonucleoprotein (RNP) and nonnucleoprotein (Sm). Anti-ENA antibodies were not found in control subjects, patients with rheumatic diseases and negative fluorescent antinuclear antibodies (FANA), or in patients with rheumatoid arthritis, dermatomyositis, drug-induced lupus, idiopathic thrombocytopenic purpura (ITP), or hemolytic anemia with positive FANA. Anti-Sm antibodies were found in 32 per cent of patients with systemic lupus erythematosus (SLE) and were not found in any other condition. There were no significant clinical or serological differences between patients with and without anti-Sm antibodies. Anti-RNP antibodies occurred in 15 per cent of SLE patients, 9 per cent of scleroderma patients, and in 100 per cent of patients with mixed connective tissue disease. SLE patients with anti-RNP antibodies had a significantly lower anti-DNA antibody titer and a significantly lower incidence of nephritis and impaired renal function. Anti-Sm and anti-RNP titers did not vary with changes in clinical status. Awareness of the presence of anti-Sm and anti-RNP antibodies is diagnostically useful. Anti-RNP antibodies have a prognostic value as well.
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PMID:The incidence and clinical significance of antibodies to extractable nuclear antigens. 30 May 68


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