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

We aimed to assess the specificity and sensitivity of (99m)technetium pyrophosphate muscle scintigraphy in the diagnostic workup of patients with suspected myopathy. We reviewed the charts of 166 patients; 52% of the subjects had myalgias, 36% had muscle weakness, 45% had an elevated serum creatine kinase (CK), and 49% had an increased C reactive protein (CRP). Scintigraphy was positive in 34 patients (20%). The test was more sensitive in the presence of muscle weakness, elevated CK, or increased CRP. The presence of myalgias did not influence the odds. Sensitivity was 60% in patients with the final diagnosis of polymyositis, dermatomyositis, or inclusion body myositis, and 70% in noninflammatory myopathies. Eight percent had false positive scintigrams. In individuals with biopsy-proven myopathy (51 subjects), the diagnostic sensitivity was 43%, and its specificity was 60%. Low positive and high negative likelihood ratios (5.0 and 0.65, respectively) document an only limited diagnostic efficiency of (99m)Tc-PYP scintigraphy in the evaluation of inflammatory and noninflammatory myopathies and suggest that the test is not helpful in the routine diagnostic workup of muscle complaints, even after a priori selection of patients for CK plus CRP abnormalities.
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PMID:99mTechnetium pyrophosphate scintigraphy in the detection of skeletal muscle disease. 1711 62

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations. The association between dermatomyositis and malignancy is becoming more clearly delineated. A sort of dermatomyositis is thought to be paraneoplastic syndrome and dermatomyositis may follow clinical course of malignancy. We report a 68-year-old woman with dermatomyositis, whose clinical onset of dermatomyositis was apparently concomitant with breast cancer. Dermatomyositis had settled down and oral steroid could be tapered after the resection of breast cancer. Creatine kinase value was elevated before the detection of the first and second recurrence and in the terminal state. At the second recurrence, skin lesions and creatine kinase value had flared up and immediate metastatic check-up revealed the recurrence. Our case shows dermatomyositis, which was thought to be paraneoplastic syndrome, that followed clinical course of malignancy and suggests immediate check-up is needed for early detection of recurrence when dermatomyositis flares up after the resection of the malignancy.
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PMID:Flare-up of dermatomyositis along with recurrence of breast cancer. 1731 65

The association between idiopathic inflammatory myositis and cancer is well recognized. Most descriptions have been of dermatomyositis-associated cancer, however, a few have been of polymyositis-associated adrenal cancer. Here, we report a 69-year-old man in whom polymyositis-associated adrenal cancer was diagnosed. The patient complained of difficulty with walking and with standing unassisted. Physical examination and electrophysiological studies revealed an abnormality of the proximal muscles. Serum levels of creatine kinase and lactic dehydrogenase were increased. Imaging studies showed a solid tumor measuring 14 x 9 cm in the retroperitoneum. After surgical excision of the tumor, including the left kidney, the serum levels of creatine kinase and lactic dehydrogenase normalized, and symptoms of myositis disappeared.
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PMID:A case of polymyositis associated with adrenal carcinoma. 1788 Feb 99

A 24-year-old Japanese woman had been suffering from a periodic fever since 10 months of age. She developed deformities in her fingers, with severe atrophy of subcutaneous adipose tissue, myositis, and frostbitten hands. She showed elevated C-reactive protein, creatine kinase, and gamma-globulin. She was also positive for antinuclear, anti-DNA, anti-SS-B, and anti-U1RNP antibodies. Her myositis was similar to amyopathic dermatomyositis rather than juvenile dermatomyositis. Although consanguineous marriage of her parents and early onset of disease suggested her disease as a hereditary disorder with periodic fever, her clinical feature and laboratory tests were unlike any known periodic fever syndromes. Her disease was regarded as a unique type of periodic-fever-syndrome-like disorder with autoimmune abnormalities.
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PMID:A case of periodic-fever-syndrome-like disorder with lipodystrophy, myositis, and autoimmune abnormalities. 1834 May 5

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.
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PMID:Lung adenocarcinoma, dermatomyositis, and Lambert-Eaton myasthenic syndrome: a rare combination. 1854 31

Kava-kava is an herbal medication, most commonly used to treat anxiety. It is derived from the roots of the pepper plant, Piper methysticum. A 47-year-old white woman presented with a rash and proximal muscle weakness 2 weeks after ingestion of kava-kava. Her creatine kinase level was elevated at 8654 U/L, and an electromyogram showed a myopathic pattern. Skin biopsy and muscle biopsy samples showed changes consistent with dermatomyositis. The patient improved with prednisone and discontinuation of the kava-kava. No similar association between kava-kava ingestion and dermatomyositis has been reported previously.
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PMID:Dermatomyositis-like illness following kava-kava ingestion. 1907 27

Rhabdomyolysis and acute polymyositis share a similar clinical profile with elevated serum creatine kinase (CK). Unlike polymyositis, the electromyography (EMG) findings of acute rhabdomyolysis with myoglobinuria are not well defined. We retrospectively evaluated 15 patients during the acute phase of rhabdomyolysis. All patients (age range, 10-84 years) underwent electrodiagnostic studies, including needle EMG, during the first 2 weeks from the onset of symptoms. All patients presented with acute myalgia, muscle tenderness, and severe generalized weakness. The median peak CK was 48.0 k u/L (25th and 75th percentiles, 11.0 and 68.0 k u/L), whereas the median CK elevation at the time of EMG examination was 10.0 k u/L (25th and 75th percentiles, 4.5 and 48.5 k u/L). Nerve conduction studies were normal in all except 1 patient who had a preexisting mild polyneuropathy. Needle EMG was performed on 117 muscles. Eighty-seven of 117 (74%) muscles were normal, and 19 of 117 (16%) showed variable proximal myopathic motor unit action potentials. None had persistent fibrillation potentials. One third of patients (5 of 15) had myopathic EMG that was present in 1 to 3 sampled proximal muscles. We conclude that the EMG findings during rhabdomyolysis are often normal and when abnormal, the changes are subtle and are in contrast to the often-prominent EMG findings in polymyositis and dermatomyositis.
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PMID:The electromyographic features of acute rhabdomyolysis. 1907 59

A 64-year-old woman presented skin lesions on her face, upper extremities and finger erythema (heliotropism and Gottron's sign). She had weakness in her lower extremities. She was given a diagnosis of dermatomyositis (DM), because the serum examination showed that a myositis-specific antibody was positive whereas Jo-1 antibody was negative. The findings of chest X-ray and computed tomography showed that she had limited small cell lung cancer, but no interstitial pneumonia. She was treated with standard chemotherapy consisting of cisplatin and etoposide with accelerated hyperfractionation radiotherapy. She showed partial response to the treatment, whereas the skin lesions and muscle weakness deteriorated accompanied with bone marrow suppression due to chemotherapy. Skin and muscle biopsy were performed and pathological findings showed typical perivasculitis infiltrated with lymphocytes in muscle and skin. With the recovery of bone marrow suppression and partial response due to chemotherapy, the skin lesions improved and creatine kinase became normalized. She was given a diagnosis of paraneoplastic DM. Since severe leukocytopenia paralleled the deterioration of DM, the decrease of peripheral white blood cell counts, especially regulatory T cell counts, may be associated with DM activity.
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PMID:[A case of small cell lung cancer with dermatomyositis that deteriorated with leukocytopenia due to chemotherapy]. 1919 12

Neuromuscular diseases accompanying thymoma include myasthenia gravis, polymyositis, dermatomyositis, and neuromyotonia. Usually 50% of patients with thymoma develop myasthenia gravis. However, only 5% show polymyositis as an accompanying paraneoplastic phenomenon. We report the case of a patient with thymoma showing myasthenia gravis as well as polymyositis. Due to the simultaneous occurrence of these paraneoplastic diseases, the criteria for exact diagnosis (serum creatine kinase, EMG, ocular involvement) overlap. This diagnostic dilemma can appreciably complicate the therapeutic approach.
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PMID:[Polymyositis associated with thymoma]. 1934 64

Chronic organizing pneumonia (COP) has often been reported as a pulmonary manifestation of collagen vascular diseases, mainly rheumatoid arthritis, but the association of COP and dermatomyositis (DM) has rarely been documented. We report a 55 year-old woman with well-documented DM and a COP. She was refractory to steroids and two other immunosuppressive agents therapy (cyclophosphamide and azathioprine). Therefore, rituximab (2 x 1 g infusions) was used for treatment. During the following weeks her strength gradually increased while creatine kinase (CK), C reactive protein and erythrocyte sedimentation rate normalized. After 6 months, she had a relapse with increased muscle enzymes, fever and moderate muscle weakness. After a second course of rituximab (2 x 1 g infusions), the patient demonstrated a remarkable clinical response as indicated by an increase in muscle strength and moderate decline in creatine kinase levels. Lung abnormalities resolved significantly on high resolution chest CT scan. Thus, B-cell depletion therapy with rituximab used alone or in combination with other immunosuppressants may be a viable option in patients with polymyositis-dermatomyositis and pneumonia refractory to current therapies.
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PMID:[Refractory dermatomyositis associated with chronic organizing pneumonia treated with rituximab: report of one case]. 1939 27


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