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

The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic diseases that include the familiar disease entities of dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). A subset of patients has unique autoantibodies which are specific for IIM (myositis specific autoantibodies; MSAs). We studied the clinical and serological characteristics of IIM in 125 Dutch patients. Sera were analysed by immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation. The most frequently encountered MSA was the anti-Jo-1 autoantibody (20%), followed by anti-tRNAHis (6%), anti-Mi-2 (6%), and anti-SRP (4%). The presence of certain MSAs was clearly associated with specific clinical characteristics. Anti-Jo-1 and anti-tRNAHis were associated with the anti-synthetase syndrome, anti-SRP with PM with severe myalgia and arthralgia and a moderate response to immunosuppressive treatment. A novel finding was the presence of anti-Mi-2, not only in DM, but also in PM. MSAs were frequently present in DM/PM sera, but were hardly ever detected in the sera of IBM patients. The few IBM patients with MSAs demonstrated a significant response to immunosuppressive treatment. It can be concluded that MSAs define specific clinical syndromes within the spectrum of IIM and that they can assist in the differential diagnosis and treatment plan of these enigmatic disorders by virtually excluding IBM by their presence, and by potentially identifying a subgroup of steroid-responsive IBM patients.
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PMID:Clinical and serological characteristics of 125 Dutch myositis patients. Myositis specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies. 1195 71

The anti-synthetase syndrome comprises the association of an inflammatory myopathy (polymyositis, dermatomyositis), interstitial pneumonitis, skin lesions characteristic of "mechanics hands", Raynaud's phenomena, inflammatory polyarthritis and, at the biological level, antinuclear antibodies known as anti-synthetases. We report our observations of two patients, one with a typical anti-synthetase syndrome and one with an incomplete form. Two men aged 49 and 47 presented with increasing dyspnoea upon effort, muscular weakness, arthralgia, bilateral pulmonary crackles and, in the first case, typical hairless skin lesions. In both cases the chest x-rays and CT scans confirmed the presence of interstitial lesions, predominantly in the lower lobes. Lung function tests showed a restrictive pattern with reduced gas transfer. At the biological level both patients presented an inflammatory picture with elevated muscle enzymes and anti-Jo-1 antibodies. Immuno-suppressive treatment with cortico-steroids and cyclophosphamide lead to a symptomatic improvement, regression of the radiological changes and improvement in the measurements of pulmonary function.
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PMID:[The anti-synthetase syndrome]. 1216 5

All 302 children treated at the rheumatology clinic of a children's hospital in Santo Domingo between September 1985 and September 1986 were included in a prospective study of the causes of joint pain in children. The 137 girls and 165 boys were grouped in five categories according to the underlying condition. The largest group, reactive arthritic conditions, affected 78 patients (25.9%). 72 of the 78 had rheumatic fever. The second largest category, hematological processes, affected 75 patients (22.6%); 40 of the 75 patients had sickle cell disease, 25 had hemophilia or other conditions, and 8 had neoplasias. 63 patients (20.8%) had infectious processes, including 33 with septic arthritis, 17 with abscesses and cellulitis, 7 with arthritis and osteomyelitis, and 6 with osteomyelitis. Prostration and pain on movement were more pronounced in patients with septic arthritis. 42 children (12.6%) had collagen vascular disease. 32 had juvenile rheumatoid arthritis, 4 had lupus, 3 vasculitis, 2 dermatomyositis, and 1 each had scleroderma and erythum nodosum. 25 patients (8.2%) had a miscellaneous array of other conditions. Sickle cell disease or neoplasia were most likely when anemia was also present. Extreme prostration and signs of inflammation suggested infectious processes. The small joints were primarily affected in juvenile rheumatoid arthritis and sickle cell disease.
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PMID:[Causes of joint pain in children]. 1229 May 50

A 36-year-old man was admitted to our hospital complaining of cough, dyspnea on exertion, skin eruptions, and joint pain. Characteristic skin lesions such as erythema around the nails, telangiectasis, and edema of the eyelids were observed in this patient. He had never complained of muscle symptoms, and his laboratory examinations showed no elevation of either CPK or aldolase. From several lines of evidence including the skin biopsy findings, amyopathic dermatomyositis was diagnosed. Chest X-ray films showed subpleural funicular opacities and consolidation in both lower lung fields. Lung biopsy specimens taken under video-assisted thoracoscopic surgery revealed nonspecific interstitial pneumonia, group II. Oral prednisolone treatment was initiated at 60 mg daily together with oral cyclosporin A (100-150 mg daily). The minimum serum concentration of cyclosporin A was maintained between 100 and 200 ng/ml. Respiratory symptoms gradually improved, and the oral prednisolone dose was tapered off. Pulmonary function and chest CT findings showed marked improvement.
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PMID:[A case of nonspecific interstitial pneumonia associated with amyopathic dermatomyositis efficiently treated with a combination of cyclosporin A and prednisolone]. 1232 32

The patient was a 58 year-old woman. Starting about seven months before the initial examination, the patient began to experience a general malaise, coupled with reddish purple scaling eruption on the MP joints, knuckles, elbows, and knees, and dark purplish erythema on both upper eyelids. About two months before the initial examination, the patient visited a local medical doctor because she began to experience arthralgia and muscular weakness. Based on the condition of her weakening proximal muscles and increased levels of myogenic enzymes, the patient was diagnosed as having Dermatomyositis. Oral administration of PSL (25 mg a day) was initiated, and the patient was referred to our department to undergo thorough examination. Although deterioration in muscular strength and elevation of myogenic enzyme levels were mild during the initial examination, the level of KL-6 was significantly elevated to 2600 U/ml. Numerous blood gas analyses and chest CT did not reveal any exacerbation of interstitial pneumonia. Various tests were performed to determine whether or not there was any malignancy, and although a small amount of ascites fluid was detected, diagnostic imaging revealed no clear abnormalities. Since cytological diagnosis of the ascites fluid was class V, an exploratory laparotomy was performed, and ovarian cancer complicated by peritoneal metastasis was discovered. As a result, the patient was diagnosed as having dermatomyositis accompanied by ovarian cancer. The level of KL-6 in the ascites fluid was significantly high at 10,900 U/ml, and immunohistological staining using anti-KL-6 antibodies confirmed the presence of KL-6 in the ovarian tumor, thus suggesting that KL-6 was produced by the ovarian cancer.
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PMID:[A case of dermatomyositis with significantly high level of KL-6 associated with ovarian cancer]. 1235 65

We present two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis. The first patient was a 38-year-old woman and the second, a 59-year-old woman. Both patients were admitted to our hospital complaining of dry cough and dyspnea on effort. The diagnosis of interstitial pneumonia was made from chest radiography, computed tomography and surgical lung biopsy. Anti-Jo-1 antibodies, which were highly specific for polymyositis and dermatomyositis (PM/DM), were detected in both patients. However, the serum creatine kinase concentrations and electromyographic findings in both patients were normal, and no clinical signs (including muscle weakness, rash and arthralgia) were found. In the first patient, oral prednisolone (PSL) treatment (20 mg day) improved the interstitial pneumonia, but PSL has now been tapered to 17.5 mg day. In the second patient, oral PSL treatment (40 mg day) improved interstitial pneumonia, and the dose was tapered to 5 mg day. The second patient was followed for more than 10 years after treatment, but she has never shown any signs of clinical myositis. Further investigation will be required, because no pathophysiological relation between anti-Jo-1 antibodies and interstitial pneumonia with PM DM has yet been established.
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PMID:[Two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis]. 1458 96

A 69 year old man complained of general myalgia, arthralgia and muscle weakness in two weeks after he started to take 10 mg per day pravastatin. Symptoms progressed at least for 3 months after withdrawal of the medication. The muscle weakness was more in distal upper extremities and the iliopsoas muscle. Serum CK was 943IU/L. The anti-nuclear and Jo-1 antibody were positive. EMG examinations disclosed fibrillation potentials at rest and myopathic discharges by a voluntary contractor. Symptoms as well as serum CK values improved promptly by treatment with prednisolone. From these clinical and laboratory results, it was speculated that this patient suffered from subacute polymyositis, although pathological studies were not performed. Because of continuing hypercholesterolemia, the patient was retreated with atorvastatin, presenting similar, but less serious symptoms. So far, three cases of statin-associated polymyositis or dermatomyositis were reported. Statins could influence the immune system, but it is still unsettled if statins cause autoimmune polymyositis. This problem warrants further study.
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PMID:[Pravastatin-associated polymyositis, a case report]. 1519 34

Calcinosis universalis is characterized by the deposit of calcium salts in skin, subcutaneous tissue, tendons and muscles. Most cases become apparent during the first decade of life. Clinical aspects may vary from arthralgia to movement limitation, with calcification of soft tissues. Differential diagnosis should exclude fibrodysplasia ossificans progressive, progressive osseous heterodysplasia, myositis ossificans and dermatopolymyositis. There is no specific treatment, but the use of calcium chelates (EDTA), biphosphonates (disodium etidronate) and steroids are mentioned. This paper presents a review of the literature and adds a new case of calcinosis universalis and its evolution in 28 months, describing laboratory and radiograph findings and suggesting the differential diagnosis among processes of soft tissue calcification.
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PMID:Calcinosis universalis: a rare diagnosis. 1593 Oct 36

Serum cytokines were evaluated in a patient with intravascular lymphomatosis (IVL). A 62-year-old man was admitted to our hospital for his general malaise, arthralgia, and high-grade fever. He was diagnosed as dermatomyositis, initially. His symptoms were improved by high dose of corticosteroid and immunosuppressive therapy. However his condition deteriorated gradually, and died ten months after the onset of symptoms. Postmortem examination revealed IVL (B cell type). The serum concentrations of inflammatory cytokines, IL-6 and TNF-alpha, were elevated. Level of serum IFN-y was high only at the end of his illness. Serum IL-2 was not detected during the course. Serum IL-4 was slightly elevated. Elevated CRP and hypoalbuminemia might be related to the elevation of these inflammatory cytokines. The clinical diagnosis of IVL is usually difficult because of the absence of specific clinical manifestations and laboratory findings. The time course and/or concentrations of serum cytokines could be a key to make an early diagnosis.
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PMID:[Patient with intravascular lymphomatosis: Changes with time in concentrations of serum cytokines]. 1657 73

Dermatomyositis is a rare inflammatory myopathy that has characteristic cutaneous lesions. Although many malignancies are associated with dermatomyositis, urogenital malignancies have rarely been reported to be associated with dermatomyositis. We report here on the first case of ureteral cancer associated with dermatomyositis. A 42-year-old man presented to us with a skin rash. A clinical diagnosis of dermatomyositis was made due to the skin lesions, muscle weakness, arthralgia, the increased erythrocyte sedimentation rate and the increased creatine kinase level. The patient revealed microscopic hematuria and abnormal urine cytology during the investigation for the underlying malignancy. Retrograde pyelography demonstrated a suspicious lesion in the right mid-ureter, and the ureteroscopic biopsy revealed the urothelial carcinoma. Although an operation was recommended, the patient died of pneumonia associated with his interstitial lung disease, which is one of the poor prognostic indicators of dermatomyositis.
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PMID:Ureteral cancer associated with dermatomyositis. 1673 68


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