Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Therapeutic results were analysed in 50 patients, including 17 with polymyositis (PM) and 30 with dermatomyositis (DM). All patients were treated with prednisone (Encorton, Polfa) and 47% of patients with PM and 73% with DM required combined treatment with prednisone and cytostatic agents, mostly with cyclophosphamide (Endoxan G.D.R.). Long-term treatment monitored with the clinical status led to remissions persisting after treatment discontinuation in 17.6% of PM patients and 24.2% of DM patients. In 64.7% of PM patients and 57.6% of DM patients regression of the clinical signs of the disease was achieved but these patients required further treatment. No information was obtained on 5 cases. Four DM patients died. The cumulative 17-year survival rate of PM patients was 100%, and that of DM patients was 78.8% . In 45 follow-up cases, the mortality was 8.9%. Side effects of the combined treatment included most frequently mucosal candidiasis (22.2%), transient leucopenia (14.8%) and recurrent respiratory tract infections (11.1%). In 5 cases liver biopsy was done and histological examination disclosed moderate fatty, glycogen and vacuolar degeneration of the hepatocytes. The statistical analysis of prognostic factors such as the diagnosis of PM or DM, sex, age at disease onset, disease duration till diagnosis establishing and beginning of treatment, fever, dysphagia, circulatory and respiratory changes, leucocytosis nad ESR at the beginning of the disease showed that sex (female), older age at disease onset and respiratory system changes may be accepted as poor prognosis factors.
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PMID:Polymyositis-dermatomyositis:25 years of follow-up of 50 patients disease course, treatment, prognostic factors. 213 29

A retrospective study of 25 patients with polymyositis-dermatomyositis (PM-DM) is analyzed with special attention to the effects of therapy and follow-up. All patients (100%) complained of muscle weakness and 68% of these demonstrated typical skin changes of DM. All patients, except 2, received corticosteroids at the onset of the disease, 23 were treated with azathioprine, 7 received cyclophosphamide, 4 methotrexate, and 1 had total body irradiation. Among the patients adequately treated with azathioprine, 75% had a good response, but 5 patients did not improve. Cyclophosphamide was used subsequently in 2, with a satisfactory response in 1. Another patient had a striking response to oral methotrexate, and total body irradiation helped to improve another patient. Although high dose corticosteroids were the preferred starting medication for the treatment of PM-DM, it is important to detect those patients who do not respond adequately and/or develop side effects. In these circumstances, the prompt use of immunosuppressive agents appears justified.
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PMID:Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment. 225 6

The treatment of the immune-mediated inflammatory myopathies remains largely empirical. Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add-on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low-dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3-6-month trial of such therapy is justified in this condition. More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms.
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PMID:Treatment of inflammatory myopathies. 914 71

A 60-year-old woman was admitted to our hospital with complaints of muscle weakness and erythema on her extremities. Gottron's sign, heliotrope rash, elevation of serum myogenic enzymes, electromyography and magnetic resonance imaging findings established a diagnosis of dermatomyositis (DM). She was treated with 60 mg of daily prednisolone. One week later, she suddenly developed splenic and renal infarctions, which were considered to have resulted from vasculopathy associated with DM. Cyclophosphamide and anticoagulants along with increasing the dosage of corticosteroid were effective. This is the first report describing splenic and renal infarctions in a patient with adult-onset DM.
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PMID:Dermatomyositis with splenic and renal infarctions during corticosteroid therapy. 1085 60

Although corticosteroids remain the mainstay of treatment for the inflammatory myopathies, their use is complicated by many side effects. Other immunosuppressive agents, alone or in combination, are being increasingly used for patients with other severe disease or treatment-related complications. Pulmonary disease remains a serious source of morbidity and mortality in myositis patients. Cyclophosphamide, cyclosporine, and tacrolimus are efficacious in patients with interstitial lung disease. Intravenous immunoglobulin is not only effective for the cutaneous complications of dermatomyositis but has been helpful in other extramuscular manifestations.
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PMID:Current approach to the treatment of polymyositis and dermatomyositis. 1109 97

Autologous stem cell transplantation (ASCT) has been proposed as a possible treatment for severe autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis (MS), systemic sclerosis, and systemic lupus erythematosus (SLE). To date, more than 250 patients with various autoimmune disorders have undergone an ASCT since 1996. Among them, there is a very limited number of children. This review summarizes the experience with ASCT for pediatric rheumatic diseases. Most reported cases concern juvenile idiopathic arthritis (JIA). Experience with ASCT for childhood SLE, Scleroderma, or Dermatomyositis is very limited. To date, 12 children with severe systemic or polyarticular JIA, all with progressive disease activity despite the use of corticosteroids, MTX, CsA, or Cyclophosphamide were treated in our center with ASCT. Rheumatologic follow-up at 3-month intervals up to 36 months showed a marked decrease in arthritis severity as expressed by the core-set criteria for juvenile chronic arthritis (JCA) activity. However, these children remain at risk for severe viral infections due to the prolonged lymfopenia. ASCT in this severely ill patient group induces a very significant and drug-free remission of the disease, but carries a significantly risk of developing fatal MAS.
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PMID:Autologous hemopoietic stem-cell transplantation for children with refractory autoimmune disease. 1112 77

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.
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PMID:[Dermatomyositis and polymyositis: clinical aspects and treatment]. 1196 87

A 46-year-old male demonstrated edematous fingers on both hands in November 2003, and interstitial pneumonia was noted on chest X-ray during a medical check-up in December 2003. Since muscular weakness and fever developed thereafter, and interstitial pneumonia was aggravated on chest X-ray and CT, the patient was admitted to our hospital on March 25, 2004. Heliotrope-like erythema, and Gottron's sign were noted. Laboratory findings showed the following ; LDH 876 U/l ; CK 224 U/l ; CRP 5.68 mg/dl ; and KL-6 3270 U/ml. Autoantibodies such as anti-Jo-1 antibody were all negative. Chest X-ray and CT showed ground-glass opacity in the bilateral lower dorsal regions of the lung, and reduced volume of the inferior lobe. He was diagnosed as having dermatomyositis (DM) associated with progressive interstitial pneumonia. Although a combination of steroid pulse therapy and Cyclosporin-A were administered, the pulmonary lesions became aggravated. Additional intravenous Cyclophosphamide (IVCY) was initiated on the 6th hospital day, and interstitial pneumonia was markedly improved. Cases of progressive interstitial pneumonia associated with DM that are negative for anti-Jo-1 antibody and show a low ratio of CK/LDH are resistant to various treatments. Our case suggested that combination therapy with steroid, Cyclosporin-A, and IVCY is useful for the treatment of progressive interstitial pneumonia with DM.
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PMID:[A case of interstitial pneumonia associated with dermatomyositis effectively treated with cyclosporin-A and cyclophosphamide pulse]. 1599 78

Systemic sclerosis (SSc) is an autoimmune disorder with a high mortality rate from pulmonary manifestations, such as interstitial lung disease and pulmonary hypertension, seen at autopsy in 90% and 75% of patients, respectively. Bronchiolitis obliterans with organizing pneumonia (BOOP) has numerous causes, but it has only been reported in three patients with SSc. We present three cases of biopsy-proven BOOP in patients with scleroderma spectrum disease, including diffuse cutaneous SSc, mixed connective tissue disease with SSc features, and limited cutaneous SSc that later evolved to dermatomyositis/SSc overlap. BOOP and SSc alveolitis have similar findings on spirometry, radiographs, and BAL; the definitive diagnosis of BOOP was therefore made by open lung biopsy. Cyclophosphamide is the current treatment of alveolitis associated with SSc, whereas high-dose prednisone is used to treat BOOP. As a result of this difference, we emphasize the importance of a thorough investigation of pulmonary disease in SSc patients to exclude BOOP and treat appropriately.
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PMID:Bronchiolitis obliterans with organizing pneumonia associated with scleroderma and scleroderma spectrum diseases. 1704 64

We described a 44-year-old man developing dermatomyositis (DM) and nephrotic syndrome (NS). Renal biopsy revealed diffuse proliferative glomerulonephritis (DPGN) with depositions of immunoglobulin and complements. A combination therapy of steroid and cyclophosphamide (CTX) was found very effective for the patient. Chronic glomerulonephritis is rare in DM. In our review of related literature, membranous glomerulonephritis (MN) is the main type of glomerular lesion, another type is mesangial proliferative glomerulonephritis (mesPGN). Here we reported a case of DM associated with DPGN developing NS, which was not found in existing literature. Although glomerulonephritis is uncommon in patients with DM, renal pathology is not as simplex as previously thought, and treatment with steroid or/and cytotoxic drugs is favorable for prognosis.
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PMID:Diffuse proliferative glomerulonephritis associated with dermatomyositis with nephrotic syndrome. 1953 41


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