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

A Caucasian male developed florid dermatomyositis documented by serum enzyme elevation, electromyography, and histology of skin and muscle. Serum enzymes, including creatine phosphokinase (CPK), aldolase, glutamic oxaloacetic transaminase (SGOT), and lactic dehydrogenase (LDH), decreased initially during high dose systemic corticosteroid therapy, although profound muscle weakness persisted. Subsequent elevation of serum LDH and SGOT levels during treatment provided a clue to underlying neoplasia. Primary hepatoma with widespread metastases was found at necropsy.
 ...
PMID:Aberrant serum enzyme patterns in dermatomyositis associated with hepatoma. 18 84

Myoglobin was detected in the sera of patients with dermatomyositis, polymyositis, scleroderma, and systemic lupus erythematosus (LE) with active myopathy. Overall, myoglobinemia was detected in 74.1% of sera taken from patients with active myositis before therapy, with slightly greater frequency in the groups with dermatomyositis and polymositis. With steroid therapy, this frequency fell to 43.4% and to 9.5% in patients in clinical remission not requiring therapy. Serum enzyme (creatine phosphokinase, lactic dehydrogenase, and SGOT) activity was higher in samples containing myoglobin, but there was considerable overlap between those with and without myoglobinemia. Sequential serum determinations in six patients demonstrated rapid reduction in the levels of serum myoglobin with therapy, usually before enzyme values had returned to normal. In one patient followed up for 30 months, myoglobinemia correlated with clinically observed exacerbations of rash and weakness to a greater degree than did enzyme determinations.
 ...
PMID:Myoglobinemia in inflammatory myopathies. 57 36

A 19-year-old girl suffering from active dermatomyositis was given suxamethonium 60 mg during anaesthesia for termination of pregnancy. A prolonged suxamethonium action occurred which was explained by the finding of homozygous atypical plasmacholinesterase in her blood. Although no fasciculations were seen immediately after injection of the drug, a period of fasciculations progressing from the extremities to the head and trunk occurred during recovery of muscle tone. No hyperpyrexia or elevation of serum creatine phosphokinase occurred. This was ascribed to the steroid therapy she received. Plasma from four other patients suffering from dermatomyositis was also investigated and one young woman, also pregnant, was found to be heterozygous for the atypical enzyme.
 ...
PMID:Dermatomyositis, suxamethonium action and atypical plasmacholinesterase. 62 8

Twenty five patients with idiopathic myositis attended this department for long term follow up from 1980 to 1989. Twelve patients had primary polymyositis (four men, eight women) and six had primary dermatomyositis (three men, three women); five women had an overlap syndrome. Two patients had a malignant condition associated with the myositis. The mean age at diagnosis was 40 years. All of the patients had proximal muscle weakness, 18/25 had a raised creatine kinase value (mean 2325 IU/l), 19/20 had an abnormal electromyogram, and 19/24 had positive muscle biopsy samples. Of the disease specific antibodies, anti-Jo-1 was detected in only 1/21 patients tested (three patients with fibrosing alveolitis were negative for this antibody), but the 56 kDa antibody was detected in 12/17 patients. The HLA data analysed in the white patients (17/25) showed that 6/8 of those tested were HLA-DR3 positive. All patients were treated with prednisolone and azathioprine was used for 14/25 patients. Only three deaths occurred during the eight year follow up, but there was a substantial morbidity, which may reflect the referral pattern. Muscle strength tests and creatine kinase levels were useful in recording the response to treatment in some patients. These data emphasise that careful long term follow up of patients with myositis is mandatory and that although the present treatment strategy has substantially reduced the death rate, morbidity associated with the disease remains a major problem.
 ...
PMID:Idiopathic myositis: a rheumatological view. 154 36

Polymyositis (PM) and dermatomyositis (DM) are dysimmune diseases usually treated with corticosteroids and immunosuppressants. Human polyvalent immunoglobulins administered intravenously (IgIV) are known to be effective in some dysimmune diseases. Between August 1987 and September 1989 we conducted an open trial of IgIV in 15 patients (mean age 44 +/- 14 years) with either PM (12 cases) or DM (3 cases) associated with a collagen disease in 2 patients. In 14 of these 15 patients the conventional treatments (corticosteroids, immunosuppressants, plasmapheresis, total body irradiation, lymphopheresis) had failed. One patient was seropositive for picornavirus and received IgIV as initial treatment. IgIV infusions were given 4 +/- 3.9 years on average after the onset of PM or DM. Twelve of the 15 patients received another treatment, starting at least 6 weeks before IgIV and pursued without dosage increase, which consisted of corticosteroids (11 cases), methotrexate (5 cases) or plasmapheresis (1 case). Human polyvalent immunoglobulins for intravenous use were prescribed in doses of 2 g/kg/monthly course. All but two patients (1 course) received 3 to 6 courses on average. The IgIV infusions were well tolerated in 12 patients; 3 patients showed allergic manifestations which regressed. Therapeutic effectiveness was evaluated by muscle testing and by repeated assays of creatine phosphokinase (CPK). Clinical improvement, usually perceptible after the first course, was observed in 13/15 patients; it was associated with a more than 30 percent decrease of the initial CPK level in 13 patients and with a reduction of associated therapies in 9 patients. In the entire patient population a statistically significant lowering of mean CPK value was observed as early as in the first course (P less than 0.001). In view of their effectiveness, rapid action and safety, intravenous Ig infusions may be regarded as an interesting treatment in PM or DM patients.
 ...
PMID:[Effectiveness of intravenous immunoglobulins in polymyositis and dermatomyositis. An open trial in 15 patients]. 170 60

The recent delineation of a clinical syndrome marked by eosinophilia, myalgia, and scleroderma-like skin changes associated with L-tryptophan use has necessitated the Centers for Disease Control to initiate a health alert. The likely association of L-tryptophan ingestion with a syndrome that mimics eosinophilic fasciitis (Shulman's syndrome) further identifies an environmental agent associated with an inflammatory sclerosing rheumatic disease process. In this report, we present the clinical, morphologic, and enzyme histochemical findings in muscle, skin, and fascia biopsies from 14 cases fulfilling the Center for Disease Control diagnostic criteria for L-tryptophan-associated eosinophilia-myalgia syndrome. The clinical syndrome reveals a high incidence of arthralgia, elbow contracture, and clinical neuropathy. The absence of significant change in creatine kinase or sedimentation rate allows for diagnostic separation from other inflammatory myopathies. Histoenzymatic features in muscle biopsies reveal a preferential epimysial-perimysial noneosinophilic infiltration characterized by acid phosphatase reactive histiocytosis, nonnecrotizing venulitis, perineural inflammation within dermis and perimysium, type II fiber atrophy with superimposed denervation features, and perifascicular alkaline phosphatase reactivity representing early neofibroplasia. The constellation of changes in skin, fascia, and muscle, with the defined clinical syndrome, allows for accurate differentiation from allied syndromes, including eosinophilic polymyositis, scleroderma, idiopathic polymyositis/dermatomyositis, polyarteritis nodosa, and toxic oil syndrome. Accurate differentiation from eosinophilic fasciitis still rests on a history of L-tryptophan ingestion.
 ...
PMID:Neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: a histomorphologic analysis of 14 patients. 198 74

A correlation study was performed on the degree of muscle weakness in 36 patients with dermatomyositis and 69 with polymyositis in relation to muscle biopsy findings, electromyography (EMG) abnormalities, and serum concentrations of creatine kinase (CK), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) enzymes. Statistically significant correlations were found between muscle weakness and EMG results in patients with polymyositis, and between muscle weakness and serum CK and AST levels in dermatomyositis. As expected, correlations were found between the results of the three enzyme determinations in both groups of patients.
 ...
PMID:Correlation between tests of muscle involvement and clinical muscle weakness in polymyositis and dermatomyositis. 208 50

Cases of polymyositis or dermatomyositis in which levels of all the serum muscle enzymes are within the normal range throughout the entire clinical course are very rare. It has recently been suggested that when there is no increase in creatine kinase the prognosis in dermatomyositis is poor and there is a higher incidence of malignancy and interstitial lung disease associated with the condition. We describe a rare case of dermatomyositis without increase in serum muscle enzymes, including creatine kinase. No evidence of malignancy or interstitial lung disease was found after a followup of 18 months. In accordance with the report of a similar case, we suggest that the absence of an increase in creatine kinase in dermatomyositis is not necessarily a poor prognostic sign.
 ...
PMID:[Dermatomyositis associated with normal serum muscle enzyme levels]. 274 49

During the last several years, there has been increasing interest in the use of intravenous immune globulin for immunosuppression. Although the mechanism(s) of action remains to be delineated, immune globulin therapy has been shown to be effective in some antibody-mediated disorders. In Rh disease, antibody-induced cytopenias, myasthenia gravis, and the clotting disorder associated with anti-factor VIII antibody, intravenous immune globulin has had therapeutic benefit. It was of interest that intravenous immune globulin may partially ameliorate the tendency toward dilation of the coronary vessels in Kawasaki disease. If this disorder represents a vasculitis of the small feeding vessels of the coronary arteries, could this agent influence other forms of collagen vascular disease? Pilot studies in dermatomyositis and polymyositis and systemic juvenile rheumatoid arthritis indicate benefit from intravenous immune globulin therapy. In these studies we used a high-dose protocol consisting of 1 gm/kg/day of immune globulin for 2 days every 4 weeks. In patients with either myositis or systemic juvenile rheumatoid arthritis, beneficial effects were seen. In the former group of patients, increased proximal muscle strength and reduction in creatine kinase levels were observed. In the latter group of patients a marked reduction in systemic features was observed. The amount of corticosteroids required was reduced in both groups of patients. These studies indicate the potential for intravenous immune globulin in collagen vascular disorders and the need for carefully controlled trials of this form of therapy.
 ...
PMID:The use of intravenous immune globulin in collagen vascular disorders: a potentially new modality of therapy. 279 2

Serum muscle enzyme levels are usually elevated in patients with untreated polymyositis and dermatomyositis. Creatine kinase is the muscle enzyme most often used to diagnose inflammatory myopathies. Seven patients with dermatomyositis and normal creatine kinase levels are described. Five of the seven patients had either an associated malignancy or severe interstitial lung disease. The one-year survival of the six patients followed for that length of time was 33 percent. A lack of creatine kinase elevation in patients with dermatomyositis is a poor prognostic sign.
 ...
PMID:Dermatomyositis without creatine kinase elevation. A poor prognostic sign. 300 9


1 2 3 4 5 6 7 8 9 10 Next >>