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

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

A prospective open label trial of add on therapy with intravenous immunoglobulin (i.v.Ig) was carried out in 16 patients with inflammatory myopathy who had continued to deteriorate or had relapsed on conventional therapy. The response was assessed using isometric myometry, functional scales, MRC grading, and serum creatine kinase concentrations with a three month run in period before commencement of i.v.Ig. Five of seven patients with isolated dermatomyositis or polymyositis and all four patients with an overlap syndrome responded to i.v.Ig with partial or complete remission of disease and normalisation of serum creatine kinase concentrations. None of five patients with inclusion body myositis showed any functional improvement although myometry scores improved in some muscles in one case. It is concluded that i.v.Ig is an effective therapeutic option in patients with drug resistant polymyositis or dermatomyositis. However, further controlled trials are required to confirm the efficacy of this form of treatment and to establish optimal doses and administration regimes.
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PMID:Immunoglobulin therapy in inflammatory myopathies. 966 70

Serum levels of carboxyterminal propeptide of type I procollagen (PICP) and aminoterminal propeptide of type I procollagen (PINP) have been used as indices of collagen synthesis in patients with various fibrotic diseases during the active stages. One of the suggested contributory factors to the development of tissue fibrosis is a decrease in collagenase activity, which may be related to levels of serum tissue inhibitors of metalloproteinases-1 (TIMP-1). In this study, the serum levels of PICP and PINP in 20 patients with dermatomyositis and 29 control subjects and of TIMP-1 in 29 patients with dermatomyositis and 29 control subjects were measured using an enzyme-linked immunosorbent assay (ELISA) or a radioimmunoassay (RIA). We found that the mean PICP level in patients with dermatomyositis was significantly higher than that in normal controls (mean +/- SD 326+/-76 ng/ml vs 135+/-88 ng/ml; P < 0.001). In 60% of dermatomyositis patients, the serum PICP level was elevated (more than 311 ng/ml, i.e. 2 x SD above the mean control value). Elevated serum PICP levels were correlated with the incidence of elevated serum creatine kinase levels in patients with dermatomyositis. The serum concentration of PINP was not elevated in comparison with that of the normal control subjects (mean +/- SD 36+/-30 ng/ml vs 63+/-34 ng/ ml). The mean TIMP-1 level in the patients with dermatomyositis was also significantly higher than in the normal control subjects (mean +/- SD 438+/-328 ng/ml vs 163+/-63 ng/ml; P < 0.001). In 59% of dermatomyositis patients, the mean serum TIMP-1 level was elevated (more than 289 ng/ml, i.e. 2 x SD above the mean control value). Serum PICP and TIMP-1 levels might be useful for detecting disease activity or severity in dermatomyositis.
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PMID:Serum concentrations of carboxyterminal propeptide of type 1 procollogen and tissue inhibitor of metalloproteinase 1 in patients with dermatomyositis. 968 76

Polymyositis and dermatomyositis (PM/DM) without creatine kinase (CK) elevation shows a poor prognosis. PM/DM is complicated with interstitial lung disease (ILD), some of which progress rapidly. To clarify the clinical features of PM/DM from the viewpoint of ILD progression, the clinical data of 25 PM/DM patients with ILD were reviewed. They were classified as responders or non-responders. The patients whose ILD responded to steroid therapy and elicited good clinical courses were termed as responders. On the other hand, the patients who had rapidly progressive ILD resistant to steroid therapy were considered as non-responders. The patients diagnosed to have DM were likely to be steroid-resistant. The non-responder group revealed significantly high aspartate aminotransferase (AST), low CK, low white blood cell (WBC), and low absolute lymphocyte counts in their peripheral blood. High CK/AST may be a favorable predictor of the disease. The percentages of lymphocytes in bronchoalveolar lavage fluid were increased in both groups. However, the percentages of two responders with low CK/AST were lower than those of three non-responders. A steroid-resistant ILD group with PM/DM may be clinically different from a steroid-responsive ILD group.
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PMID:Clinical features of polymyositis/dermatomyositis with steroid-resistant interstitial lung disease. 974 52

A 59-year-old woman with dermatomyositis suffered pneumomediastinum twice during steroid therapy for aggravated myositis. In both instances, pneumomediastinum occurred when the patient's aggravated myositis was in remission. Plain chest X-ray films showed a slit-like air lucency around the left pulmonary artery. This indicated that air entered the mediastinum through the left hilum. Although pneumomediastinum associated with dermatomyositis is considered to be fatal, in our patient each instance of pneumomediastinum spontaneously disappeared very quickly. The favorable course of our patient seemed to be associated with her high elevated levels of serum creatine kinase and seropositive results for anti-Jo-1 antibody.
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PMID:[Recurrent pneumomediastinum in the course of interstitial pneumonia associated with dermatomyositis]. 991 85

Juvenile dermatomyositis (JDM) is characterized by microvasculopathy of the striated muscle, which indicates different etiology, clinical manifestation and prognosis from the adult-onset dermatomyositis. We experienced 10 cases of JDM and 1 case of juvenile polymyositis (JPM) in the recent 14 years, and analyzed clinical manifestation, laboratory findings, treatment anrognosis. The cases were 9 girls and 2 boys. The onset of the disease was 2 years of age in 2 patients, and 9 to 13 years of age in 9 patients. During the follow-up courses, no cases were dead or complicated with neoplasm. Skin rash was the most frequent manifestation at the onset, and facial erythema was common. Muscle weakness was observed only in 4 cases at the onset, and in all cases muscle enzymes including creatine kinase and aldolase were elevated. The clinical course was classified into three groups; monocyclic (5 cases), chronic and recurrent (4 cases), and fulminant (2 cases). Prognosis depended not on the degree of the elevated serum muscle enzymes, but on the initial therapy employed at the onset of the disease. Five cases including 2 cases of fulminant type were initially treated with methylprednisolon pulse therapy, and all of these had no recurrence. On the other hand, 6 cases were started the therapy with p.o. prednisolone. Four of them had frequent recurrences in accordance with tapering of prednisolone. These cases were effectively treated with the combination with immunosuppressants. In previous reports, JDM and JPM were reported to be a disorder which had relatively favorable prognosis. But we found that one third of the cases had chronic and recurrent courses. Methylprednisolone pulses as initial therapy may be effective in preventing the chronicity and recurrence of the disease.
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PMID:[Clinical analysis of 11 cases of juvenile dermatomyositis and polymyositis]. 1004 16

The clinico-laboratory features of 16 patients with dermatomyositis (DM) were compared between patients with accompanying rapidly progressive interstitial lung disease (RP-ILD, n = 7) and those with chronic interstitial lung disease (C-ILD, n = 9), and also between deceased (seven RP-ILD and three C-ILD) and living patients (six C-ILD). The extent of muscle weakness of the extremities and frequency of autoantibody positivity were significantly lower in DM patients with RP-ILD than in DM patients with C-ILD. Furthermore, significantly lower serum creatine kinase/lactate dehydrogenase levels (0.26+/-0.27) were found in the 10 patients who died than in the six living patients (1.21+/-1.09). A higher CD4+/CD8+ T-lymphocyte ratio in the peripheral blood (3.51+/-2.65) was detected in the four DM patients with RP-ILD who died than in the six living DM patients with C-ILD (1.22+/-0.49).
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PMID:Clinico-laboratory characteristics of patients with dermatomyositis accompanied by rapidly progressive interstitial lung disease. 1063 71

An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.
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PMID:High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels. 1069 94

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
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PMID:Dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. 1071 98

Thirty-six patients with juvenile dermatomyositis, seen consecutively between 1983 and 1996 and treated initially with low doses of corticosteroids (prednisolone 1 mg/kg/day), were studied retrospectively to evaluate their long-term evolution and to identify factors predictive of the functional outcome. After a mean follow-up of 4.9 years, 28 (78%) of the patients were well without functional impairment; five patients had inactive disease but with persisting disabilities; and three patients had active disease despite several years of treatment. Fifteen children (42%) developed dystrophic calcifications which, in five of these patients, interfered with functions. These patients treated for juvenile dermatomyositis with a low dose corticosteroid regimen had an evolution identical to that of the published series of patients treated with higher doses and probably had a better quality of life. The best predictors of good functional recovery and minimal calcinosis were early treatment after the onset of symptoms and low creatine kinase serum level at the time of diagnosis.
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PMID:Childhood dermatomyositis: clinical course of 36 patients treated with low doses of corticosteroids. 1072 93


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