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

This is the first report on idiopathic inflammatory myopathies (IIM) in French Canadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4, 13%), anti-U1RNP (n = 3, 10%), and anti-Ro (n = 2, 7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1 autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
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PMID:Distinctive features of idiopathic inflammatory myopathies in French Canadians. 887 Jan 12

We encountered a patient with dermatomyositis complicated by sarcoidosis. A 57-year-old woman was admitted to our hospital because of fever dry cough, and myalgias. There were reticular shadows on her chest X-ray film. Although the typical skin rash and myositis suggested the diagnosis of dermatomyositis biopsy specimens from a salivary gland, muscle, and lung revealed noncaseating granulomas as well. Uveitis was also noted. These findings suggested the coexistence of sarcoidosis with dermatomyositis. Examination of the lung-biopsy specimens showed interstitial pneumonia compatible with dermatomyositis, except for the granuloma. The typical rash of dermatomyositis and pathological findings of the lung specimen were inconsistent with sarcoidosis. Therefore we concluded that this patient had both dermatomyositis and sarcoidosis. This case sheds new light on the importance of pathological examinations.
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PMID:[Dermatomyositis complicated by sarcoidosis]. 897 83

Dermatomyositis (DM) has been associated with gastrointestinal malignancy but not with polyps or adenoma. We report a case of villous adenoma associated with DM. An 80-year-old white woman was referred for a rash of 2 months' duration that was suggestive of DM. On examination, Gottron's papules and heliotrope rash were seen. Muscle strength was 4/5 in the proximal upper and lower extremities. Despite normal muscle enzyme values, electromyographic and nerve conduction studies were supportive of the diagnosis of DM. DM was confirmed by deltoid muscle biopsy. Heme-positive stool was seen on rectal examination. Colonoscopy revealed a large tubulovillous adenoma in the cecum, which was resected. The patient was given oral prednisone, 60 mg/day. Within 4 weeks after surgery, steroids were tapered to one third the original dose. Within 9 months, steroid therapy had been discontinued with no evidence of disease activity.
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PMID:Dermatomyositis in association with tubulovillous adenoma: resolution after resection of adenoma. 907 5

The association of leukocytoclastic vasculitis or dermatomyositis with malignancies has been reported. We describe a patient who developed a skin rash, histologically compatible with dermatomyositis, which during the course of the disease switched to leukocytoclastic vasculitis, which was accompanied with peripheral blood pancytopenia in the absence of any specific pathological manifestation from the bone marrow three years prior to the diagnosis of acute myelomonocytic leukemia (AMML).
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PMID:Facial heliotrope rash as the initial manifestation of acute myelomonocytic leukemia. 916 50

Dermatomyositis is an inflammation of the striated muscle with an important leukocyte infiltrate which is accompanied by a characteristic cutaneous exanthema. In the international classification we find DM associated with neoplasm in 10% of cases. It is accepted that neoplasms are related to DM if it does not exceed it in two years (prior o after the onset of the disease. The most commonly associated are lung, ovary and breast cancers. In the pas ten years only two cases of DM associated to renal cancer (both in renal cell cancers) have been published. In this article we present a case history of a woman with a DM associated to a renal oat-cell carcinoma. Also, we will review the literature on this theme and will evaluate the predictive parameters of the presence of malignancies in this pathology.
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PMID:[Dermatomyositis and small cell tumor of the kidney]. 918 52

Inasmuch as the clinical features of the idiopathic inflammatory myopathies are not easily differentiated from those of other similar rheumatic and neurologic conditions, diagnosis is often difficult. Various classification criteria for polymyositis and dermatomyositis have been suggested by a number of investigators. The most commonly accepted and used criteria include symmetric proximal muscle weakness, serum elevations of muscle enzymes, the classic electromyographic and muscle biopsy findings of inflammatory myopathy, and the typical skin rash of dermatomyositis. Although these criteria are clinically useful, they can result in misdiagnoses and inappropriate therapies. They also result in heterogeneous patient groups being selected for clinical and laboratory studies. Furthermore, they do not include recent findings related to the myositis-specific autoantibodies and magnetic resonance imaging of muscle that have been found to be important adjuncts in assessing patients with muscle weakness or elevations of muscle enzymes. A modification to the Bohan and Peter criteria is proposed to include myositis-specific autoantibodies and magnetic resonance imaging. This proposal could initiate productive discussions and investigations of the sensitivity and specificity of new classification criteria for myositis and could ultimately enhance our treatment capabilities.
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PMID:Classification criteria for the idiopathic inflammatory myopathies. 937 82

A patient (47-year old female) who had erythema similar to Gottron's sign on bilateral finger joints since two years ago, started to have polyarthralgia on bilateral knee and shoulder on the spring of 1993. Polyarthralgia was extended to both wrist and hand joints on Oct. of 1995. On the middle of Dec. 1995, she began to have exertional dyspnea and was referred and admitted in our hospital on 18th, Dec., 1995. Chest X-ray and CT scan showed the shadow for active interstitial pneumonitis on bilateral lower lung fields. Blood gas analysis indicated hypoxia (PaO2: 62.8 mmHg) and low % DLCO (64.7%). Skin eruption of face (heliotrope-like erythema) and hands (Gottron's sign) and skin biopsy (right hand) findings were compatible with that in dermatomyositis. The analysis in blood biochemistry showed no elevation for muscle enzymes. The diagnosis for amyopathic dermatomyositis (ADM) was made according to the criteria proposed by Euwer & Sontheimer (1993). The steroid pulse therapy and 50 mg per day of cyclophosphamide were immediately administered. The dyspnea and dermatitis were improved within two weeks after therapy. She is presently in remission until Jan. 1997 with the maintenance dosis of 10 mg per day of oral prednisolone.
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PMID:[A case of amyopathic dermatomyositis associated with interstitial pneumonitis]. 939 75

Interstitial pneumonia complicated with dermatomyositis sometimes shows a resistance to high dose steroid therapy and a fatal course particularly in patients without showing the elevation of creatine kinase. We experienced a 48 year old woman who developed heliotrope rash, Gottron's sign, multiple cutaneous ulcers, and dyspnea on exertion. These symptoms were resistant to low dose steroid therapy. Serum levels of creatine kinase were normal. Anti-nuclear antibodies and anti-Jo-1 antibody were negative. High resolution CT scan of the chest showed areas of multiple air space consolidation and subpleural linear shadows. Lung biopsy performed under video-assist thoracosurgery revealed diffuse alveolitis with scattered lymphoid folicules and mild accumulation of macrophages in the alveolar spaces. There were no honey-combing. These features were compatible with "non-specific interstitial penumonia" proposed by Katzenstein, 1995. The patient was treated with 10 micrograms lipo-PGE1, PGE1 incorporated in lipid microspheres, and 300 mg pentoxifylline, which resulted in a dramatic improvement of both interstitial pneumonia and cutaneous ulcers. The present case suggested a novel strategy for the treatment of interstitial pneumonia.
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PMID:[Successful treatment of interstitial pneumonia with lipo-PGE1 and pentoxifylline in a patient with dermatomyositis]. 939 76

Cutaneous manifestations of dermatomyositis (DM) commonly include Gottron's papules, cuticular erythema and telangiectasia, periorbital edema with a "heliotrope" rash, a papulosquamous eruption of the hairline, face, and trunk, as well as poikiloderma. Very few references can be found concerning vesicular and bullous lesions, however. We present two patients with dermatomyositis who manifested vesicular and bullous lesions who were initially misdiagnosed. Although rare, it is important to recognize this form of DM to avoid misdiagnosis. Furthermore, in some cases, vesiculo-bullous DM may portend a poor prognosis.
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PMID:Vesiculo-bullous dermatomyositis. 955 87

Classical myopathic dermatomyositis (DM) is a chronic autoimmune disease characterized by an erythematous rash and severe, proximal muscle weakness. A disease variant, amyopathic DM, presents with the typical rash but without clinical evidence of muscle weakness. Prednisone and immunosuppressive drugs alleviate symptoms in many patients. Accurate longitudinal evaluations of patients are important to limit serious side effects of these drugs, including osteoporosis, cataracts, and growth inhibition. Metabolic abnormalities detected with 31P magnetic resonance spectroscopy (MRS) provide the best quantitative data for evaluating these patients. With 31P MRS, the levels of inorganic phosphate (Pi), phosphocreatine (PCr), ATP, and phosphodiesters (PDE) were determined in the quadricep muscles of patients during rest and exercise. Artificial neural network (ANN) analyses of these data were previously used for accurate classification of patients with myopathic or amyopathic DM and normal controls. In the present investigation, an artificial neural network was employed for further analysis of the 31P metabolite levels in quantitative, longitudinal evaluations of the extent (percent) of clinical improvement or deterioration during treatment with prednisone and immunosuppressive drugs. The ANN results showed that adult patients in a severe myopathic state could improve with treatment to a clinical status of amyopathic DM. In contrast, severely weak juvenile patients in the myopathic state recovered to normal status. One juvenile patient did not improve and remained in the myopathic state. Additionally, a serious clinical relapse in an amyopathic patient was predicted with serial ANN analyses well in advance of the actual clinical event. These network analyses show potential utility for clinical applications in muscle diseases.
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PMID:Analysis of 31P MR spectroscopy data using artificial neural networks for longitudinal evaluation of muscle diseases: dermatomyositis. 971 79


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