Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 16-year-old girl, one of dizygotic twins, presented in 1976 complaining of a 1-year history of a lack of coordination and an inability to run. The results of biochemical tests confirmed the diagnosis of classic abetalipoproteinemia. In addition to the recognized neurologic features of this disorder, she had a reduced evoked motor unit potential and markedly elevated serum levels of muscle enzymes, which suggested myositis. The serum vitamin E level was markedly decreased. Oral therapy with vitamin E, 800 mg daily, was begun, and in 1981 the dosage was increased to 3200 mg daily. Over the 7 years of follow-up she improved clinically, there was an increase in the evoked motor unit potential, the serum levels of some of the muscle enzymes decreased to normal, and the serum and tissue vitamin E levels increased significantly. It was concluded that treatment with high doses of vitamin E was responsible for the arrest of the usually progressive neuropathy and myopathy.
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PMID:Arrest of neuropathy and myopathy in abetalipoproteinemia with high-dose vitamin E therapy. 298 Nov 35

The inflammatory myopathies have diverse clinical and pathological features and multiple etiologies. Some are confined to a single muscle or group of muscles (e.g., orbital myositis and localized nodular myositis) while others are diffuse. Infective forms may be due to viral, bacterial, fungal, protozoal, or parasitic organisms. Viruses may cause acute self-limited forms of myositis and have been isolated from muscle in some cases of acute rhabdomyolysis and inclusion body myositis. They have also been implicated in some cases of congenital myopathy and in polymyositis and dermatomyositis, but there is no evidence of viral invasion of muscle in these conditions. In polymyositis and dermatomyositis there are derangements in humoral and cellular immune function, and recent evidence suggests an underlying disturbance of immunoregulation. The roles of genetic factors, drugs, and Toxoplasma infection have been under scrutiny. There is increasing recognition of immunological and pathological differences in polymyositis and juvenile and adult dermatomyositis, and in cases with associated connective tissue diseases, suggesting different underlying pathogenetic mechanisms. Inclusion body myositis, eosinophilic myositis, and granulomatous myositis can be separated from the other idiopathic inflammatory myopathies because of distinctive clinical and pathological features and this may also reflect different mechanisms of muscle injury. Recent developments in the treatment of the idiopathic inflammatory myopathies include the use of plasmapheresis and total-body irradiation in cases that are resistant to corticosteroids and immunosuppressive drugs.
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PMID:Inflammatory myopathies: Part 1. 298 25

A patient with sarcoidosis who presented with acute inflammatory myopathy is described. The patient had no symptoms other than those of the muscle involvement, with elevated serum levels of creatine kinase. Muscle biopsies revealed epithelioid granulomas, consistent with sarcoidosis. There was a dramatic response to a moderate dose of corticosteroids, but relapse occurred after the dose was tapered. We suggest that sarcoidosis be considered in the differential diagnosis of inflammatory myopathy, and that patients with acute sarcoid myositis be maintained with at least a moderate dose of corticosteroids for at least 6 months.
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PMID:Sarcoidosis presenting as acute myositis. Report and review of the literature. 306 66

We describe a patient who developed a benign myopathy with features suggestive of subacute perimysial inflammation. There was no blood eosinophilia or infectious agent identified. Creatine phosphokinase was not significantly elevated. Electromyography showed a myopathic process. Muscle biopsy showed predominantly subacute perimysial inflammation with rare eosinophils. The patient recovered completely after therapy with glucocorticoids and a nonsteroidal antiinflammatory drug. The features of our case were compared to known causes of myositis with or without eosinophils. It may be part of the spectrum of relapsing eosinophilic perimyositis or may be a condition previously unreported.
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PMID:Subacute perimyositis. 318 78

Interstitial pulmonary fibrosis occurs in approximately 9% of patients with PM/DM, yet its effect on the course of PM/DM has been scarcely noted. In this report, two patients with PM/DM and IPF were presented to highlight the fact that pulmonary disease can overshadow the primary muscle disorder and progress despite therapy with corticosteroids and nonsteroidal immunosuppression. Our patients were added to previously reported cases and an overview of PM/DM and IPF was presented. Sixty-seven patients with the diagnosis of PM/DM and IPF, with a mean age of 60 +/- 18 years, were identified. Pulmonary complaints were present in 64 cases. Fever was present in 18, arthritis or arthralgias in 11, and Raynaud's phenomenon in 9. Forty percent died after followup of 31 +/- 32 months. This mortality was significantly higher (P less than .05) than that in 745 historical controls with PM/DM without IPF. Progressive IPF was the immediate cause of death in 58% of those who died. A subgroup of 29 patients who had histologic documentation of both myositis and IPF had a mortality of 62% after 22 +/- 25 months. In this latter group, six patients had RP; five of these died. Patients who were not treated with corticosteroids also appeared to fare worse but, given the small number of patients involved, definite conclusions cannot be drawn. We conclude that patients with PM/DM can be adversely affected by the presence of IPF and that this negative impact may be exaggerated by RP and perhaps by lack of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Adverse impact of interstitial pulmonary fibrosis on prognosis in polymyositis and dermatomyositis. 318 43

One hundred and fifteen cases of injection myopathy with deltoid and/or gluteal fibrotic contracture were studied, almost all of whom had definite histories of repeated intra-gluteal or intra-deltoid injections. One third had siblings affected by the same fibro-muscular disorders manifested by focal muscle atrophy and limitation of adduction and flexion of the shoulder or hip. Electromyography disclosed myopathic changes of the fibrotic muscles. Muscle biopsy showed marked perimysial and endomysial fibrosis with non-specific degeneration, regenerative changes and, in some cases, partial denervation signs. Under the electron microscope, endomysial and perimysial collagen fibrils lost their normal unimodal diameter distribution and showed a rather broad spectral distribution of diameters suggesting a defective control of collagen formation in this disease entity. Repeated injection injuries and myotoxicity resulting in multifocal myositis are the first trigger of this fibrotic syndrome, and abnormal control of collagen formation could be another important pathogenic factor.
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PMID:Syndrome of deltoid and/or gluteal fibrotic contracture: an injection myopathy. 322 1

Paraspinal muscle biopsy was done on the erector spinae muscles in sixty consecutive scoliotic patients. Together with the clinical and muscle biopsy findings, these sixty patients were classified into thirty-two patients with idiopathic scoliosis, eleven patients with myopathic scoliosis, nine patients with neuropathic scoliosis, and eight patients with miscellaneous scoliosis. Of the thirty-two patients with idiopathic scoliosis, nineteen cases displayed neurogenic changes in the grouping of the Type 1 fibers and target fiber formation more common on the convex side back muscles. The myopathies consisted of central core disease, nemalin myopathy, congenital fiber type disproportion, mitochondrial myopathy, congenital muscular dystrophy, and myositis. Patients with neuropathic scoliosis all showed loss of the normal mosaic pattern and grouping of both Type 1 and 2 fibers in their muscle biopsies. This result indicates that there is a variety of neuromuscular diseases in scoliotic patients. The neurogenic changes observed more commonly on the convex side muscle of idiopathic scoliotic patients deserve further investigation.
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PMID:Morphological studies on the erector spinae muscle in sixty consecutive scoliotic patients. 324

Infantile myositis is an inflammatory myopathy occurring in children under one year of age. This condition is extremely rare in the neonatal period and may be confused with other causes of generalized weakness. Creatine kinase activity is usually markedly elevated and electromyography demonstrates low amplitude, polyphasic motor unit activity. Muscle biopsy, necessary for diagnosis, documents characteristic findings of perifascicular atrophy and the presence of perivascular inflammatory cells. The diagnosis should be followed by corticosteroid treatment. The patient presented is the youngest biopsy-proved case of infantile myositis. In this report, his symptoms and clinical course are compared with those of previously described patients. The role of infectious agents and the immune state in the etiology of infantile myositis is considered.
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PMID:Infantile myositis: a case diagnosed in the neonatal period. 350 96

Skeletal muscle obtained from a patient with distal myopathy with rimmed vacuole formation (DMRV) was examined by a new direct labeled antibody enzyme method of immunohistochemistry. Abnormal increases of cathepsins B and H, capable of degrading the myofibrillar proteins, were demonstrated to be localized at the site of vacuoles and other intramyofibral portions and not to be associated with concomitant increases of their endogenous inhibitors. Autodigestion by these intramyofibral lysosomal proteinases may be of major importance in focal destruction of myofibers and formation of vacuoles, because no myofiber necrosis with invasion of macrophages, overt myositis, and neuropathy was seen in the muscle. These findings provide the first reasonable explanation of myofibral breakdown and atrophy in DMRV, and should be helpful in further studies on the mechanism of myofibral breakdown in various vacuolar myopathies and other myopathies of so far unknown pathogenesis.
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PMID:Abnormal increases of lysosomal cysteinine proteinases in rimmed vacuoles in the skeletal muscle. 351 18

Clinical and pathologic observations made in a patient with inflammatory myopathy associated with the acquired immunodeficiency syndrome (AIDS) are presented. Multinucleated giant cells were a prominent histopathologic feature in the muscle biopsy samples. The findings indicate that in some patients with AIDS myositis, inflammatory myopathy may be the direct result of infection with the human immunodeficiency virus.
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PMID:Myositis and acquired immunodeficiency syndrome. 359 91


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