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 13-year-old girl with chronic graft versus host disease (GVHD) developed severe proximal weakness. Muscle biopsy showed necrotic fibers with anti-IgG deposits and interstitial inflammation. Steroid treatment helped, but her weakness recurred. Repeat biopsy showed persistent inflammation and possible steroid myotoxicity. Numerical ratio of capillaries to myofibers and capillary numerical density were higher than childhood dermatomyositis (CD), and capillaries had no tubuloreticular inclusions. Absence of quantitative histologic, ultrastructural, or immunocytochemical abnormalities of capillaries differentiated our patient from CD. We conclude that myositis of chronic GVHD can present as a severe myopathy that responds to steroids and differs from CD.
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PMID:Myositis of chronic graft versus host disease. 635 Sep 24

Indirect immunofluorescence (IIF) was used to detect antinuclear antibodies (ANA) in 42 clinical cases. In each case cryostatic rat kidney slices and cultivated HEp-2 cells were used as substrates. Clinical diagnoses were as follow: Progressive Systemic Sclerosis (PSS) 25 cases, of which 8 were acrosclerotic, 8 diffuse, 5 CREST syndrome, 1 overlap PSS + Systemic Lupus Erythematosus (SLE) and 3 PSS + myopathy; Localised scleroderma (morphea): 3 cases; Mixed Connective Tissue Disease (MCTD): 3 cases; "Idiopathic" Raynaud's Disease (RD): 4 cases; Dermatomyositis (DM): 2 cases (1 paraneoplastic); SLE: 1 case; Unclassifiable Connective Tissue Disease (UCTD): 4 cases. The ANA-positive cases identified by the traditional technique were divided according to pattern into 4 categories: homogeneous, peripheral, speckled, nucleolar. In contrast those identified using HEp-2 cells were divided into 9 pattern groups: (nuclear type) centromere, fine speckled, coarse speckled, diffusely grainy, homogeneous: (nucleolar type) speckled, clumpy, homogeneous. The results demonstrated a higher general incidence of positivity with HEp-2 cells and confirmed the close connection between Anticentromere ANA and CREST syndrome. A similarly close connection was noted between MCTD and both nuclear diffusely grainy and nucleolar speckled patterns. A fairly clear connection was also noted between acrosclerotic or diffuse SSP and a fine speckled nuclear pattern. It is felt that ANA tests using IFI on HEp-2 cells should lead to significant progress in the field of diagnosis and prognosis and the study of PSS subsets.
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PMID:[Clinical significance of antinuclear antibodies in progressive systemic sclerosis]. 636 19

A 69-year-old woman with classical rheumatoid arthritis developed a severe dermato-myopathy during treatment with penicillamine. Remission occurred on withdrawal of the drug. Penicillamine (dimethylcysteine) is a pharmacological agent used for its chelating properties in the treatment of Wilson's disease and heavy metal poisoning, and in cysteinuria because of soluble disulphide formation. Within the last 17 years penicillamine has been increasingly applied in the treatment of rheumatoid arthritis, the mechanism of action still being unknown. A great number of side effects have been reported, including less common auto-immune disorders such as drug-induced systemic lupus erythematosus, myasthenia gravis and polymyositis. These and other possible side effects have been well reviewed by others (1, 2). To our knowledge only a few earlier cases of dermatomyositis as a complication to penicillamine treatment of rheumatoid arthritis have been reported (3, 4, 5). We describe here another case.
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PMID:Penicillamine-induced dermatomyositis. A case history. 665 98

Increased oxygen transport by the circulation is normally tightly coupled to increased oxygen uptake (VO2) during exercise; cardiac output (Q) increases 5 to 6 liters for every liter of increased oxygen utilization (delta Q/delta VO2 congruent to 5). We measured cardiac output and oxygen uptake at rest and during bicycle exercise in 7 patients with myalgia without evident muscle disease and 15 patients with myopathies. Resting circulation was normal in all patients, and during exercise the increase in cardiac output relative to oxygen uptake was normal in all myalgia and most myopathy patients. However, in four patients (with dermatomyositis, phosphorylase deficiency, carnitine deficiency, and ocular myopathy with "ragged-red fibers") exercise cardiac output was excessive and delta Q/delta VO2 high, resulting in an abnormally high level of cardiac work for a given level of exercise. This hyperkinetic response may represent aberrant regulation of the circulation by skeletal muscle as a consequence of some myopathies.
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PMID:Hyperkinetic circulation during exercise in neuromuscular disease. 668 23

A precipitating antigen-antibody system has been characterized that occurs in patients with polymyositis. At least half of the patients not only have polymyositis but also have scleroderma. The proposed name for this antigen found in calf thymus extract (CTE) is PM-Scl, to indicate the almost universal presence of polymyositis and the frequent occurrence of scleroderma in the patients who make antibodies to this antigen. The antigen is probably nucleolar since all sera which precipitate with the PM-Scl antigen stain the nucleoli of Hep2 cells by indirect immunofluorescence. The PM-Scl immune system is a distinctive one different from the other known precipitins that occur in patients with polymyositis and dermatomyositis including Jo1, nRNP, and Mi. This PM-Scl antigen and its antibody represent one system which constitutes part of the reactions previously designated as PM1. Interlaboratory exchange of sera and extracts have established the unique nature of this reaction which occurs in patients with inflammatory myopathy.
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PMID:Antibodies to a nuclear/nucleolar antigen in patients with polymyositis overlap syndromes. 669 38

33 patients with idiopathic inflammatory myopathies (polymyositis or dermatomyositis) and 45 of their first-degree relatives were investigated in a search for any influence of genetic factors in these diseases. None of the relatives had evidence of an inflammatory myopathy but 13 had some other autoimmune disease. Mean serum IgG levels were reduced and serum C3c concentration increased both in patients and relatives. Levels of IgM were reduced and C4 increased in some groups of patients. The incidence of autoantibodies was increased in the patient group, particularly in those with isolated dermatomyositis or other systemic features, but not in the relatives. HLA, blood group and other genetic markers showed no deviation from normal population frequencies. Evidence favouring a genetic influence on the etiology of idiopathic inflammatory myopathy is restricted to disturbance of levels of some Ig and complement components, and for these there may be other explanations.
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PMID:A search for genetic influence in idiopathic inflammatory myopathy. 695 66

Human idiopathic inflammatory myopathy is an acquired disorder with an annual incidence of two to five cases per million. A genetic influence on host susceptibility may also play a role. With the marked heterogeneity of the disease one of the major challenges is to identify subsets that might share a more uniform pathogenesis and manifest a less diverse profile of clinical findings, histopathological abnormalities, and natural history. Dermatomyositis can be distinguished by clinical appearance and pathological changes, but the recognition of additional disease subsets remains very inexact. Current evidence suggests that dermatomyositis occurs as a result of a vasculopathy, but immune mechanisms involved in other categories of idiopathic inflammatory myopathy may also involve cell-mediated immunity and possibly multiple mechanisms. Even though viral-induced muscle inflammation occurs in humans, there is no convincing evidence for a viral cause of idiopathic inflammatory myopathy. Experimental allergic myositis may be produced by the injection of animals with skeletal muscle homogenates and complete Freund's adjuvant, but the myositogenic factor is unknown and the parallels between experimental allergic myositis and human idiopathic inflammatory myopathy are limited.
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PMID:Inflammatory myopathy: a review of etiologic and pathogenetic factors. 698 34

Childhood dermatomyositis is a distinct subset of dermatomyositis with highly variable outcome. We reviewed our experience with 29 patients observed over 22 years and attempted to correlate tissue manifestation with outcome. Distinctive vascular lesions included non-necrotizing vasculitis and a unique spectrum of endovascular injury producing temporary or permanent occlusion of small arteries and capillaries. Vessels with noninflammatory endovasculopathy were often reactive with fluorescein-conjugated antisera to IgM, C3d, and/or fibrin. Lesions linked to endovascular injury include infarction, zonal myopathy, and loss of capillary network. We were able to identify half of the children destined to have persistent morbidity on the basis of severity of vasculopathy in pretreatment muscle-biopsy specimens. Our observations support a central role for endothelial cell injury in the pathogenesis of childhood dermatomyositis, suggest a basis for assessing the efficacy of therapy, and provide a focus for investigation of basic mechanisms.
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PMID:Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. 706 42

A 46-year-old woman had heliotrope erythema, poikiloderma, and proximal muscle weakness. Histologic examinations of the skin were consistent with dermatomyositis. Muscle biopsy revealed numerous noncaseating granulomatous lesions within the muscle fasciculi, confirming the diagnosis of sarcoid myopathy. No granulomatous lesion could be detected clinically in other organs. A combination of this skin and muscle disorder is rare and has not been reported previously.
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PMID:Sarcoid myopathy with typical rash of dermatomyositis. 719 3

A 2-year study was undertaken which identified 60 patients who had an abnormal electromyogram (EMG), muscle biopsy, and/or clinical examination suggestive of myopathy. The clinical records were reviewed, and 12 patients were found to fulfill the criteria of Bohan and Peter for definite diagnosis of polymyositis or dermatomyositis. Electromyography was performed on distal and proximal muscles including the paraspinals on 1 side of the body. Biopsies were taken contralaterally from muscles determined to be abnormal from electrodiagnostic tests. The most significant finding was the degree of involvement of the paraspinal musculature. These muscles showed 1 to 4 plus spontaneous potentials in all 12 patients. Volitional recruitment was early and complete. Mean action potential duration and amplitude decreased, and polyphasia increased. Comparing the degree of electrodiagnostic abnormalities in different muscle groups revealed sole involvement of paraspinals in 1 patient and predominant involvement in 8. Confirmative biopsies were taken from paraspinal muscles of 3 patients. In 1 patient, the biopsy of the paraspinal muscle was positive for myositis while the biceps brachii muscle biopsy proved to be equivocal. Our study emphasizes the EMG and histologic significance of the paraspinal musculature in the diagnosis of idiopathic inflammatory myopathy.
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PMID:Electromyographic and histologic paraspinal abnormalities in polymyositis/dermatomyositis. 723 95


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