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Results for “DERMATOMYOSITIS”

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At least 307 records · Page 17Linked to original sources
PubMed1992

"Catch-up" growth in steroid dependent dermatomyositis treated with cyclosporin-A.

OBJECTIVE: To improve the short-term growth, and hopefully final height attainment, of children with dermatomyositis treated with chronic daily corticosteroid regimens. METHODS: We have examined the growth of nine pre-pubertal children (7F, 2M), mean age 8.9 (range, 5.7-12.3) years, who had severe juvenile dermatomyositis and required persistent corticosteroid therapy. All were treated with cyclosporin-A. RESULTS: During a mean period of 1.3 years (range 1.0-1.7) cyclosporin-A treatment was associated with a marked decrease in the dosage of prednisolone, from a mean of 12.4 to 6.6 mg/day (p < 0.02), and an increase in height velocity standard deviation score from a mean of -1.37 to +1.39 at 6 months (p < 0.02) and -0.33 at one year. During the first year of treatment with cyclosporin-A, seven patients were able to either withdraw or reduce their prednisolone dose. No side effects of treatment were observed. CONCLUSIONS: Cyclosporin-A was a useful treatment modality in juvenile dermatomyositis and allowed a decreased dose of corticosteroids and associated "catch-up" growth.

Body Height↗
PubMed1992

[Dermatomyositis as paraneoplastic syndrome of bladder tumor].

The dermatomyositis is a connective tissue disease characterized by changes affecting both the skin and the muscle, appearing most frequently around the fifth and sixth decades and which association to a vesical tumour is very rare. This paper contributes one case of paraneoplastic dermatomyositis associated to infiltrant vesical tumour, presenting erythematous damage in face, nape of the neck and upper thorax, as well as periorbital heliotrope erythema and fingernails base and sides telangiectasia, all of which are typical signs of dermatomyositis.

Aged↗
PubMed2003

[Amyopathic dermatomyositis].

Dermatomyositis represents a progressive autoimmune disease of the connective tissue with SLE-like dermatitis, with inflammatory myopathy and muscular weakness. It is an infrequent and serious disease affecting not only the skin and striated muscle. Cases without skin affection are classified as polymyositis. Beside autoimmune mechanisms, aetiology includes also genetic, environmental and paraneoplastic factors. Clinical skin symptoms as well as histopathological findings include Gottron's papulae, heliotropic periorbital erythrema, which, together with palpebral oedema, brings about the weepy look of patients. None of these signs is pathognomic and beside the cutaneous symptoms the diagnosis requires 3 out of 4 other diagnostic criteria for the muscle symptoms. Cutaneous symptoms can precede myositis with various time intervals; such situation is sometimes classified as Amyopathic dermatomyositis (ADM), dermatitis sine myositis or skin dermatomyositis. A case of an ADM patient and the analysis of problematic including the strategy of therapy are presented.

Dermatomyositis↗
PubMed1992

[Interstitial fibrosing pneumopathy and dermatomyositis. Pulmonary histology and prognosis].

The authors describe a case of interstitial lung disease associated with dermatomyositis. Alveolar-interstitial shadowing on the initial chest X-ray, lymphocytosis on the broncho-alveolar lavage fluid, absence of anti-JO1 antibodies and presence of intra-alveolar buds on the open lung biopsy were observed. Our patient died of acute respiratory failure, 34 months after symptoms onset. Intra-alveolar buds found at an early stage of dermatomyositis interstitial lung disease, were associated with minimal interstitial cellularity, markedly interstitial fibrosis, poor prognosis and poor response to steroid therapy. In contrary, bronchiolitis obliterans organizing pneumonia noted at an early stage of interstitial lung disease associated with both dermatomyositis and polymyositis, seems to have better prognosis and response to corticosteroid, especially if associated with polymyositis.

Bronchoalveolar Lavage Fluid↗
PubMed2005

[Bucillamine-induced dermatomyositis-like clinical features in a patient with rheumatoid arthritis].

A 78 year-old woman was admitted to our hospital because of subacutely progressive dysarthria, dysphagia, proximally dominant muscle weakness and erythema in the neck and back. She was diagnosed as having rheumatoid arthritis (RA) at the age of 60 and treated with bucillamine (BUC) for 8 years. Laboratory tests included a rheumatoid factor of 1,472U/ml. Serum creatine kinase level was slightly elevated. The activated T cells in the peripheral blood were markedly increased. Needle EMG demonstrated myogenic changes. The magnetic resonance image of the left upper arm showed diffuse muscle atrophy and inflammatory changes in the triceps muscle. The muscle biopsy revealed perivascular inflammatory cell infiltraton and type II fiber atrophy. A biopsy from the skin showed mild perivascular inflammatory cell infiltraton. According to the results of these findings, she was thought to have dermatomyositis due to BUC. After withdrawal of BUC followed by the administration of prednisolone 1mg/kg, her symptoms improved and activated T cells in the peripheral blood were decreased. In Japan, BUC is widely accepted as an effective drug in the treatment of RA, even though it is known to induce some autoimmune diseases. However, the mechanism of the development of autoimmune disease is unclear. We considered that the long-term use of bucillamine could trigger an autoimmune response such as an increase in activated T cells and the development of dermatomyositis-like clinical features in our patient. In conclusion, when RA patients treated with BUC show a clinical picture compatible with dermatomyositis, its causative relationship has to be considered.

Aged↗
PubMedDate not supplied

Regression of cutis calcinosis with diltiazem in adult dermatomyositis.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

Calcinosis↗
PubMed2005

[A case of corticosteroid-resistant nonspecific interstitial pneumonia associated with dermatomyositis successfully treated with cyclosporin A].

We report a case of corticosteroid-resistant nonspecific interstitial pneumonia associated with dermatomyositis. The patient was successfully treated with a combination of cyclosporin A (CsA) and prednisolone. A 28-year-old man was admitted complaining of skin eruptions, myalgia, and weakness, and dyspnea on exertion. Characteristic skin lesions such as erythema around the nails, teleangiectasis, and edema of the eyelids were observed. Dermatomyositis was diagnosed based on the diagnostic criteria, including the skin biopsy findings. Laboratory examination showed elevation of creatine kinase, but the anti-Jo-1 antibody was negative. Chest X-ray on admission showed reticular shadows and airspace consolidation mainly in both lower lung fields. Lung biopsy specimens taken under video-assisted thoracoscopic surgery revealed infiltration of lymphocytes into the thickened alveolar septa, and this revelation was compatible with the description of cellular nonspecific interstitial pneumonia. Administration of corticosteroids with steroid pulse therapy resulted in with improvement of interstitial pneumonia. However, after the combination therapy of corticosteroid with daily oral administration of 100-150 mg of CsA had been initiated, hypoxemia recovered swiftly in 5 days, and respiratory symptoms improved gradually, but steadily. Oral administration of corticosteroid and CsA was eventually tapered off and terminated completely 5 years later. The patient has been quite well with no treatment for two and a half years. The case presented here implies that CsA combined with corticosteroid may be efficacious in the treatment of interstitial pneumonia associated with dermatomyositis which is refractory to corticosteroid therapy.

Adult↗
PubMed2005

[Non invasive mechanical ventilation in intensive care unit in patient with respiratory failure as the first manifestation of dermatomyositis].

Polymiositis and dermatomyositis are idiopathic inflammatory myositis with several respiratory complications but the acute failure respiratory is uncommon, and in the more severity types needs noninvasive mechanical ventilation. The case report a dermatomyositis patient who needed intensive care to failure respiratory due to muscle weakness who did not respond to corticoids -with pulsed prednisolone- in combination with another immunosuppressive agent with methotrexate, and after this with cyclofosfamide and cyclosporin. Along her stay in ICU is used a succesfully treatment with noninvasive mechanical ventilation and added high dose immunoglobulin intravenous, although there is a controversial about its use, was an effective treatment due to drug resistant dermatomyositis front to immunosuppression.

Adult↗
PubMed2005

Juvenile amyopathic dermatomyositis.

A 3-year-old girl presented with a 6-month history of multiple, light-pink, flat-topped papules over the dorsal aspects of the metacarpophalangeal and interphalangeal joints of the hands and feet. Nailfold telangiectases, ragged cuticles, and a heliotrope color of the upper eyelids were also evident, but there was no clinical evidence of muscle weakness and levels of muscle enzymes were normal. A biopsy specimen from one of the papules showed a vacuolar interface dermatitis consistent with a diagnosis of dermatomyositis. This report draws attention to juvenile amyopathic dermatomyositis, which is an uncommon subtype of dermatomyositis with an excellent prognosis.

Child, Preschool↗
PubMed2006

[Clinical analysis of pneumomediastinum complicated in polymyositis and dermatomyositis].

OBJECTIVE: To analyze the clinical features of pneumomediastinum complicated in polymyositis and dermatomyositis (PM/DM) and to study the pathogenesis thereof. METHODS: The clinical data of 4 patients with pneumomediastinum complicated in dermatomyositis out of 447 PM/DM patients hospitalized in Peking Union Medical College (PUMC) Hospital Jan 1989 to June 2005, were analyzed. The records of patients with PM/DM available in English throughout the world were reviewed to collect those with pneumomediastinum as a complication. And the data of these patients were analyzed, focusing mainly on the age, gender, peak of serum creatine kinase (CK), presence of pneumomediastinum, cutaneous vasculopathy, chest radiographic changes, tracheal cannula, management, and outcome. RESULTS: Among the 447 patients with PM/DM hospitalized in PUMC Hospital, 134 males and 313 females, aged 42 +/- 17, pneumomediastinum was observed as a complication in four patients, 3 males and 1 female, aged 12 - 43, with a prevalence rate of 0.9%. Together with 17 cases reported in the English literatures there were 21 patients with pneumomediastinum complicated in polymyositis and dermatomyositis (PM/DM in all. Only one of the literatures reported a prevalence rate as high as 8.3% (4/48), and other literatures were merely case reports. Compared with the PM/DM patients without pneumomediastinum the mean age of the PM/DM patients with pneumomediastinum was significantly younger (34:42, P < 0.01), the male: female ratio significantly higher (13:8 to 132:311, P < 0.01), the morbidity rates of interstitial lung disease and of cutaneous vasculopathy significantly higher (18/21 to 134/443, and 12/21 to 44/443, both P < 0.01). Although statistic analysis could not be undertaken because of the peak of CK not being provided in details in the literatures, the CK levels of the patients with pneumomediastinum were mostly normal or mildly higher with a peak lower than 500 U/L Three of the 4 patients with pneumomediastinum hospitalized in PUMC Hospital and 5 of the 443 patients (1.1%) without this complication received tracheal cannula. There was a significant association of pneumomediastinum with tracheal cannula (P = 0.000). CONCLUSION: Vasculopathy is strongly suspected as being responsible for the pneumomediastinum in DM, and male gender, younger age, interstitial lung disease, and tracheal cannula may be the risk factors of this pneumomediastinum complicated in PM/DM.

Adolescent↗
PubMed2006

Recurrent episodes of dermatomyositis-associated pneumonitis masquerading as hypersensitivity pneumonitis.

This case report describes a unique presentation of dermatomyositis-associated pneumonitis. A 44-year-old man presented with repeated episodes of fever, dyspnea, and hemoptysis accompanied with pulmonary infiltrates, on chest CT scan. Hypersensitivity pneumonitis was suspected. Further work-up showed clinical and serologic evidence of dermatomyositis-associated pneumonitis. The patient was treated with oral prednisone and azathioprine. The subject of dermatomyositis-associated pneumonitis is discussed.

Adult↗
PubMedDate not supplied

[Clinico-endoscopic and morphometric characteristics of gastric and duodenal mucosa in children with dermatomyositis].

A total of 53 patients with dermatomyositis aged 4-14 with the disease duration of three months to eight years were under observation. Twenty seven patients complained of stomach-ache, II felt pain during palpation, 30 lost appetite. Electrogastrography revealed disorders in bioelectric activity of the stomach muscles in 99.9% of the patients. Endoscopic studies revealed lesions in the stomach and duodenum in all the patients, in 39 of them gastroduodenitis in the stage of exacerbation was diagnosed, in 7 erosion, in 3 peptic and duodenal ulcer. In 81.3% of the children during clinico-laboratory remission of dermatomyositis gastroduodenitis persisted at the stage of exacerbation. The vessels lying in the mucosa of the antral area of the stomach and duodenum underwent peculiar changes: arterial walls looked thickened, nuclei of the endothelial cells were enlarged, rounded up. One could observe infiltrates around the vessels consisting mainly of lymphocytes. Morphometric indices of the mucosa were also different in the antral area of the stomach and duodenum. Apparently, changes revealed in the gastric and duodenal mucosa of children with dermatomyositis occur due to vascular processes developing as a result of the main disease. This should be taken into consideration in the treatment of such children.

Adolescent↗
PubMed1991

Vasculitis and calcinosis in juvenile dermatomyositis.

Dermatomyositis of childhood onset is characterized by vasculitic lesions and often complicated by calcinosis. We describe 32 patients with juvenile dermatomyositis. All suffered from vasculitic skin changes like facial erythema often with edema, Gottron's sign, telangiectasias, erythematous eruptions, different rashes and necrotic ulcerations. Vasculitis appeared also in inner organs as gastrointestinal ulceration, neurologic and cardiac manifestation. 4 children complained of Raynaud's phenomenon. Calcinosis of soft tissues developed in 21 patients within 0.5 to 10 years after onset. In 6 of them we saw regression of calcium deposits after a progressive phase of 1 to 5 years. Functional outcome in juvenile dermatomyositis depends mainly on the degree of calcinosis together with shortening of diseased muscles.

Adolescent↗
PubMedDate not supplied

[Cellular immunity and the autoantibody system in polymyositis and dermatomyositis].

Polymyositis and dermatomyositis are clinical forms of inflammatory muscle diseases of unknown etiology. Cellular immunity seems to have great importance in pathogenesis of the idiopathic inflammatory myopathy. It is well known that specifically sensitised lymphocytes from the peripheral blood of patients with polymyositis and dermatomyositis may secrete various mediators (e. g. lymphokines) and be cytotoxic to muscle cell tissue culture as well. In recent years, a variety of anti-muscle antibodies and antibodies to nuclear and cytoplasmic antigens have been identified in the serum of patients with idiopathic inflammatory muscle disease, but their role in this clinical syndrome is still unknown. In this review article authors bring out some of the current state of knowledge about polymyositis and dermatomyositis related autoantibody systems, including biochemical characteristics of the target antigens, epidemiological and clinical significance, and possible role of these autoantibodies in the development of the disease.

Autoantibodies↗
PubMed1991

[Dermatomyositis--diagnostic value of capillary microscopy].

The results of conventional capillary microscopy and fluorescence videomicroscopy are described in 7 patients with well established dermatomyositis (mean age 34.3 +/- 19 years, mean duration of the illness 25.7 +/- 26.9 months). All patients showed marked microangiopathy characterized by avascular fields, increased capillary tortuosity and enlargement, and enhanced transcapillary diffusion. The capillary microscopy pattern was not specific for dermatomyositis. Nevertheless, capillary microscopy plays an important role in the differential diagnosis of this condition. It remains unclear how sensitive this method is in the early detection of dermatomyositis.

Adult↗
PubMed1989

[Spectrum of antinuclear and anti-cytoplasmic antibodies in dermatomyositis and polymyositis overlap syndromes].

Antinuclear antibodies (ANA) of the IgG class were detected in 60% of patients with dermatomyositis. Only in 16% could we demonstrate precipitating antibodies against the nuclear Mi-2 antigen. These antibodies are considered to be serological markers of dermatomyositis. The ANA spectrum in polymyositis-overlap syndrome was considerably more heterogeneous: patients with polymyositis/progressive systemic scleroderma overlap had antibodies against the nucleolar PM-Scl antigen or the nuclear Ku antigen. Cytoplasmic antibodies to Jo-1 were present in polymyositis associated with Sjögren's syndrome and pulmonary fibrosis. Antibodies against ribosomal ribonucleoprotein were found in polymyositis with systemic lupus erythematosus and antibodies to nuclear ribonucleoprotein in polymyositis associated with mixed connective tissue disease. The investigation demonstrates that the characterization of ANA specificities helps to differentiate between dermatomyositis and distinct forms of polymyositis-overlap syndrome.

Adult↗
PubMed1989

[Polymyalgia rheumatica with drug eruption--an important differential diagnosis to dermatomyositis].

With regard to certain clinical features, polymyalgia rheumatica (PR) may closely resemble dermatomyositis. In contrast to dermatomyositis, PR usually does not show any cutaneous manifestations, although there might be seen concomitant giant cell arteritis. Furthermore, we do not find muscle enzymes in the serum with PR, and there is no histologic evidence of myositis. In rare cases, however, PR may be associated with cutaneous drug eruption and/or non-specific increase of muscle enzymes, which might cause considerable difficulties regarding the diagnostic differentiation from dermatomyositis.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1989

[Anesthetic management of a patient with dermatomyositis--clinical observation of the effect of muscle relaxants].

A 58 year old woman suffering from dermatomyositis underwent elective surgery for spinal caries. Concerning the anesthetic management of patient suffering from dermatomyositis, there is little information on the appropriate use of muscle relaxants. It is generally suspected that the patient is sensitive to nondepolarizing muscle relaxants. Anesthesia was with oxygen-nitrous oxide and fentanyl. Pancuronium 6 mg was given intravenously after awake intubation and an additional dose of 2 mg was given after 7.3 hours. During anesthesia neuromuscular function was monitored by neuromuscular transmission monitor (Datex Relaxograph). Duration of neuromuscular block was defined as the time for the twitch height to recover from total paralysis to 25% of the control value. Duration in this patient was 3.1 hours and this was longer as compared with the values of 1.1-1.8 hours obtained in 7 control patients. It is suggested that a usual dose of muscle relaxants results in a relatively higher effect in the patients with dermatomyositis because of their diminished muscle mass. The anesthetist should be careful in using muscle relaxants. The muscle relaxants should be given to such a patient with monitoring closely the neuromuscular function using a neuromuscular transmission monitor.

Anesthesia↗