Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A now 20-year-old man was first diagnosed as having dermatomyositis when aged 12 years. At that time he had muscular weakness and heart failure followed a year later by heterotopic calcifications. For over seven years he was treated with corticoids, at times also with azathioprine or methotrexate. Nonetheless the calcifications progressed. Because of the extensive calcifications, predominantly of the extremities, he is severely disabled and has been confined to a wheel-chair. For one year he was treated with EHDP (1-hydroxyethylidenediphosphonate), up to 16 mg/dl per day. In addition he received prednisolone (10 mg/d) and azathioprine (100 mg/d). But radiologically there has been further slight progression of the calcifications, indicating that EHDP has been ineffective in this patient.
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PMID:[Extraosseous calcification in juvenile dermatomyositis. The ineffectiveness of EHDP]. 313 11

Few guidelines exist for the use of corticosteroid therapy in polymyositis-dermatomyositis (PM-DM). We retrospectively examined the relationship between serum creatine kinase (CK), muscle strength and the dosage and method of administration of prednisone in 30 patients with PM-DM observed monthly for a minimum of one year. Forty-two corticosteroid treated episodes of proximal muscle weakness associated with CK elevations formed the final study group. Each patient course was designated as having a good or poor biochemical and clinical outcome based on predetermined criteria. Adherence to 3 principles predicted a favorable biochemical and clinical outcome in the treatment of myositis: (1) administration of an adequate initial (loading) corticosteroid dose; (2) continuation of the initial dose until or after the time that the serum CK had become normal; and (3) a slow corticosteroid taper rate. Achievement of a CK within the low normal range predicted a prolonged biochemical remission, and a rise of CK within the normal range signalled a subsequent biochemical and clinical relapse. Tapering the corticosteroid dose when the CK was elevated frequently resulted in a further increase in CK. These observations allowed us to develop practical guidelines for the management of PM-DM.
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PMID:Relationship between serum creatine kinase level and corticosteroid therapy in polymyositis-dermatomyositis. 317 94

Five patients with dermatomyositis and 2 with polymyositis between 3 and 12 years old are reviewed. All of them fulfil the Bohan and Peter diagnostic criteria. Five presented misery before weakness. Two presented acute renal failure. The pathologic muscular study was not always specific of inflammatory myopathy and without correlation with the degree of symptoms. Treatment which prednisone and in one patient also azathioprine resulted with complete remission in 4 patients.
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PMID:[Idiopathic inflammatory myopathies in childhood. A study of 7 patients]. 319 70

Muscle strength determined as torque was measured in patients with dermatomyositis and polymyositis. Patients demonstrated weakness in both quadriceps and hamstring muscle groups. The ratio of quadriceps to hamstring torque (Q:H) was 1.93 +/- 0.38 in controls and 1.60 +/- 0.62 in patients. Patients with Q:H ratios that were below this mean were older, weaker, and were treated with greater doses of prednisone. Fifty-six percent of patients studied initially had low Q:H ratios, and 64% of all patients had low Q:H ratios at some time. The Q:H ratio in patients with steroid and thyroid myopathy, however, was not different than controls. It is hypothesized, therefore, that preferential involvement of the quadriceps group characterizes patients with inflammatory muscle disease.
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PMID:Abnormalities in quadriceps-hamstring strength. Relationships in polymyositis and dermatomyositis. 323 May 63

Patients with agammaglobulinemia are particularly susceptible to chronic enteroviral infections of the central nervous system. Data on 42 patients were obtained by literature review, communications with other physicians, and personal experiences. Thirty-eight patients had congenital immunodeficiencies, most frequently X-linked agammaglobulinemia. Most patients who could be assessed were receiving maintenance therapy with intramuscular gamma-globulin before their enteroviral infection. Seven patients had not been recognized as hypogammaglobulinemic before the onset of infection. The commonest pathogens were echoviruses (37 of 41 cases), especially type 11 (11 cases). Thus far, four patients have had sequential or simultaneous infections with a second enteroviral serotype. Other features of the disease have included weakness, lethargy or coma, headaches, hearing loss, seizures, ataxia, and paresthesias. Some patients have also had nonneurologic manifestations of chronic enteroviral infection, including fever, the dermatomyositis-like syndrome, edema, rashes, and hepatitis. Treatment has consisted primarily of antibody administration, either in intravenous immunoglobulin preparations or in immune plasma. Twelve patients have received intraventricular immunoglobulin through reservoir devices; six of these 12 have improved substantially, as judged by clinical criteria.
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PMID:Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. 329

Muscle and joint pains and/or weakness are not usually stressed as central symptoms in hypothyroidism. Two cases of long-standing hypothyroidism presenting with prominent myopathic symptoms are described. The first patient presented with a 12-year history of proximal myopathy, arthropathy and skin abnormalities, and florid primary myxedema was diagnosed. No evidence for a systemic autoimmune process was found. The second patient had been treated with irradiation to the neck 23 years before admission and presented with clinical and laboratory signs of both proximal myopathy and hypothyroidism. Thyroid hormone replacement resulted in a complete recovery of all the musculoskeletal symptoms, with reversion to normal of the very high muscle enzyme levels in both patients. The cases presented illustrate that hypothyroidism can lead to the development of a variety of muscular, rheumatic and dermatologic syndromes easily confused with dermatomyositis or other collagen diseases.
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PMID:Musculoskeletal symptoms as a presenting sign of long-standing hypothyroidism. 343 93

Polymyositis is an autoimmune, inflammatory disease affecting human skeletal muscle. In the presence of concomitant vasculitis in the skin, the term dermatomyositis is used. In contrast, systemic lupus erythematosus (SLE) is a multisystem disease in which involvement of the skin, kidneys, joints, brain, and other organs may be found. The clinical manifestations vary according to the organ/system involved. It is clinical and therapeutic importance to define which organ/system is involved during the course of the disease. We approached this problem by studying the specificity of autoantibodies that are generated in patients with SLE and polymyositis/dermatomyositis. Among such antibodies are those directed against nuclear components including a variety of ribonucleoprotein (RNP) complexes. We have utilized mammalian nuclear preparations enriched with RNP particles as the antigenic source for immunoblotting studies to identify specific antigenic polypeptides. In the study reported here, sera from five groups of patients were examined: 10 patients with dermatomyositis/polymyositis; six patients with SLE and myositis; 12 lupus patients with cerebral and/or renal disease; eight patients with SLE but no myositis, renal, or cerebral disease; and 5) 11 patients with muscle weakness or muscle disease not due to myositis. In the first two groups of patients with myositis, antibodies against a nuclear RNP protein of 56 KD was identified in 12 of 16 sera. In contrast, such antibodies were found in the serum of only two of 20 patients with SLE but without muscle involvement (groups 3 and 4), and were not found at all in patients with other muscle diseases. This study has identified a new marker, antibodies against a nuclear RNP protein of 56 KD for detecting muscle involvement among the autoimmune rheumatic diseases.
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PMID:Autoantibodies against a specific nuclear RNP protein in sera of patients with autoimmune rheumatic diseases associated with myositis. 349 67

Sixteen cases of dermatomyositis-polymyositis in children (dermatomyositis 15; polymyositis 1) have been reviewed. Proximal muscle weakness with a characteristic skin rash was vital for diagnosis in the majority of children. The positive laboratory tests such as creatine phosphokinase (CPK), electromyography (EMG) and muscle biopsy were helpful but results were normal in some cases even in the acute phase of the illness. All but one had been treated initially with high dose corticosteroids followed by a low dose maintenance for a prolonged period. Two in addition had had cytotoxic agents. Thirteen of the sixteen were currently in remission. In the other three cases, one with polymyositis only, the disease remained active and the two with dermatomyositis died. Only three of the patients were incapacitated by residual fixed contractures and extruding calcinosis. On the whole the prognosis of dermatomyositis is good.
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PMID:Dermatomyositis-polymyositis in children. 351 51

A 10-year-old girl from southern Alberta, Canada, who had close contact with cats, developed typical features of dermatomyositis. The diagnosis was confirmed by muscle biopsy. A toxoplasmosis titer was 1:16,384 by indirect fluorescent antibody technique, and the IgM response to toxoplasma was positive. Only minimal improvement followed prednisone and azathioprine administration, but she rapidly improved after 4 weeks of treatment for toxoplasmosis with pyrimethamine and sulfadiazine. A year after the onset of dermatomyositis, she showed no weakness or cutaneous lesions, and a repeat muscle biopsy no longer showed inflammation, perifascicular atrophy, or regeneration of myofibers. She remains asymptomatic more than 2 years after discontinuation of all medications. Investigation for immune deficiency disease 1 year after therapy revealed that lymphocytic response to T-cell and B-cell mitogens was normal, as were immunoglobulin and complement levels. She had mild impairment of natural killer cell activity and a positive antinuclear factor. Her rapid improvement on specific therapy and lack of significant long-term immune deficiency is consistent with acute toxoplasmosis infection in an immunologically competent child.
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PMID:Juvenile dermatomyositis induced by toxoplasmosis. 359 37

We describe a 14-month-old child with dermatomyositis in whom calcinosis was the first sign of the disease. This case shows that calcinosis, usually a late complication of dermatomyositis, may be the presenting sign of the disease even in young children and when Gottron's sign is still absent and muscular weakness not prominent.
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PMID:Calcinosis as the presenting sign of juvenile dermatomyositis in a 14-month-old boy. 369 83


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