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)

Antiglycyl tRNA synthetase is an unusual autoantibody specificity associated with polymyositis and dermatomyositis complicated by interstitial lung disease. We report here autoantibodies to glycyl tRNA synthetase in a patient with systemic lupus erythematosus and interstitial lung disease. During the course of her disease, the patient developed elevated muscle enzymes and worsening pulmonary function. A quantitative immunoprecipitation technique was developed to evaluate the relationship between autoantibody production and clinical manifestations in this patient. Increasing serum antiglycyl tRNA synthetase antibody levels correlated with the severity of the patient's interstitial lung disease and with the level of creatine phosphokinase.These results suggest that certain autoantibodies, such as antiglycyl tRNA synthetase, might reflect an underlying pathologic process (in this case, myositis and interstitial lung disease) irrespective of disease diagnosis, and that quantitative immunoprecipitation may be a useful technique for investigating the relationship between specific autoantibody production and organ involvement in systemic autoimmune disease, Quantitation of these autoantibodies may be useful clinically in monitoring disease activity and/or response to therapy.
 ...
PMID:Correlation of antisynthetase antibody levels with disease course in a patient with interstitial lung disease and elevated muscle enzymes. 1907 36

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.
 ...
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.
 ...
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.
 ...
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.
 ...
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.
 ...
PMID:[Refractory dermatomyositis associated with chronic organizing pneumonia treated with rituximab: report of one case]. 1939 27

A 74-year-old patient, suffering for 20 years from polycythemia rubra vera, was treated with hydroxyurea for 2 years. Intensive xerosis (ichthyosis-like) and violaceous papules on the dorsal hand surfaces were observed. There was also reddish purple periorbital erythema resembling "heliotrope erythema". Such a disease pattern has been described as "hydroxyurea-induced dermatomyositis-like eruption" - a drug-adverse reaction associated with hydroxyurea therapy. The reaction has typical dermal features of dermatomyositis without any systemic symptoms (in the case of our patient, antinuclear antibodies absent and creatine phosphokinase within the normal range). The above reaction is associated with chronic hydroxyurea uptake and, after withdrawing the drug, alleviation of skin lesions usually takes place after 10 days to 18 months with no recurrences. In the case of our patient, we observed clinical improvement after 3 months; xerosis decreased, heliotrope erythema vanished, and there was also a decrease in both the number and intensity of violaceous papules on the dorsal hand surfaces.
 ...
PMID:Dermatomyositis-like eruption induced by hydroxyurea: a case report. 1978 27

We report patients from two neuromuscular centers who were evaluated between the years 2000 and 2008 and met the following criteria: (1) proximal muscle weakness occurring during or after treatment with statins; (2) elevated serum creatine kinase (CK); (3) persistence of weakness and elevated CK despite discontinuation of the statin; (4) improvement with immunosuppressive agents; and (5) muscle biopsy showing necrotizing myopathy without significant inflammation. Twenty-five patients fulfilled our inclusion criteria. Twenty-four patients required multiple immunosuppressive agents. Fifteen patients relapsed after being tapered off immunosuppressive therapy. Exposure to statins prior to onset was significantly higher in patients with necrotizing myopathy (82%) as compared to those with dermatomyositis (18%), polymyositis (24%), and inclusion-body myositis (38%) seen in the same time period. The lack of improvement following discontinuation of statins, the need for immunosuppressive therapy, and frequent relapse when treatment was tapered suggest an immune-mediated etiology for this rare, statin-associated necrotizing myopathy.
 ...
PMID:Immune-mediated necrotizing myopathy associated with statins. 1981 88

A man in his late fifties was treated with acitretin for psoriasis. The treatment was discontinued when liver enzymes started to increase. He subsequently developed erythrodermia, weakness of proximal muscle groups, heliotrope rash, Gottron's papules and elevation of creatine kinase to more than 9000 U/L. However, histological examination of a muscle biopsy displayed neuropathic atrophy and only a few scattered necrotic fibers. Alpha 1 foetoprotein was substantially elevated (1600 kU/L) and computed tomography showed two hepatic tumors, lytic lesions in the spine and in the ninth rib. Although no chronic liver disease could be documented, the patient was found to have a hepatocellular carcinoma which presented as dermatomyositis and erythrodermia.
 ...
PMID:[A man with erythrodermia, muscle weakness and weight loss]. 1989 74

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.
 ...
PMID:[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]. 1999 56


<< Previous 1 2 3 4 5 6 7 8 9 10