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)

The folic acid antagonists, aminopterin and then methotrexate, have been the primary systemically administered drugs for the treatment of severe psoriasis for 25 years. Methotrexate was finally approved by the FDA 5 years ago and was recently estimated to be used for approximately 25,000 psoriatic patients annually. With the small but chronic doses used for psoriasis, methotrexate has been relatively safe, except for a limited occurrence of significant hepatotoxicity. The good to excellent results obtained in most patients must, therefore, be weighted against the small risk of liver damage. Methotrexate has also been used with some effectiveness in several other dermatologic conditions, including mycosis fungoides, dermatomyositis, pityriasis rubra pilaris, and others, although they are not yet FDA-approved indications. The recent successful introduction of photochemotherapy (psoralen and ultraviolet light) for moderate and severely affected psoriatics will probably lead to a substantial decrease in the number of patients requiring methotrexate.
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PMID:Methotrexate. 31 25

Methotrexate is now used widely for the treatment of acute leukemia, non-Hodgkin's lymphoma, osteogenic sarcoma, choriocarcinoma, breast carcinoma, pulmonary and epidermoid carcinoma, and intrathecal chemotherapy. It is also useful in bone marrow transplantation, severe psoriasis, rheumatoid arthritis, dermatomyositis, Wegener's granulomatosis and sarcoidosis. The recent dramatic intensification of methotrexate therapy can be attributed in part to advances in our understanding of the clinical pharmacology of the folate antagonists, as well as to the combination of positive results and their effective dissemination to medical oncologists. The review summarizes the pharmacologic findings and illustrates how they are currently being applied to the treatment of malignant disease.
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PMID:The clinical pharmacology of methotrexate: new applications of an old drug. 34 86

The inflammatory myopathies encompass a group of heterogenous muscle diseases which have in common an acquired myopathy with histological signs of endomysial inflammation. We present evidence based on recently emerged clinical, histologic, immunopathologic, demographic and therapeutic observations that these myopathies comprise three major and distinct groups: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM). Immune-mediated mechanisms characteristic for each group appear to play a primary role in the pathogenesis of these diseases. In DM there is an intramuscular microangiopathy mediated by the C5b-9 membranolytic attack complex, leading sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis and perifascicular atrophy. In contrast, in PM and IBM the muscle fiber injury is initiated by sensitized CD8+ cytotoxic T cells that recognize MHC-I restricted muscle antigens, leading to phagocytosis and fiber necrosis. Among the viruses implicated in the cause of inflammatory myopathies, only the retroviruses, HIV, HTLV-1 and simian retroviruses, have been convincingly associated with PM. Retroviruses, therefore, appear to be the leading group of viruses capable of triggering these diseases. The treatment of inflammatory myopathies has been largely empirical. A detailed therapeutic plan based on our experience with a large number of patients is presented. Patients with bona fide PM or DM respond to steroids to some degree and for some period of time. In contrast, patients with IBM do not respond to any therapy and the disease should be suspected when a patient with presumed PM has failed treatment. Methotrexate and cyclophosphamide are disappointing. Cyclosporine and Azathioprine are commonly used but they are of uncertain benefit. Plasmapheresis is ineffective. High-dose intravenous immunoglobulin is a promising new therapeutic modality.
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PMID:Clinical, immunopathologic, and therapeutic considerations of inflammatory myopathies. 142 35

Thirteen cases of dermatomyositis seen in children are described. Records of hospitals serving 250,000 children were reviewed for the 10-year period during which these children were seen and no other cases were found. Eleven of the 13 are living, including seven believed to be in complete remission; the remainder have ongoing disease of varying severity. Literature concerning this unusual childhood disease is reviewed and there is general agreement as to the characteristic nature of the clinical picture. Special features, including rash, muscle findings, deformity, calcinosis and gastrointestinal bleeding, are discussed. Emphasis is placed on the value of steroid therapy, though some serious complications are acknowledged. Methotrexate, found promising by others, was without benefit in two cases.
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PMID:Juvenile dermatomyositis. 432 24

We describe five cases of polymyositis/dermatomyositis with interstitial lung disease, occurring in women, with a mean follow up of 28 months (15 to 51). One remained without respiratory symptoms, two had a chronic and relapsing course, and two an acute course, leading to death with respiratory failure in one. Anti-Jo1 antibody was found in two patients, without relation to the gravity of the respiratory illness. The two others with a severe course had anticardiolip antibodies, and one cutaneous necrosis of the fingers. Cancer-associated dermatopolymyositis was never seen at diagnosis, but a lymphoma of the brain was diagnosed after two years in one patient. Combination of corticosteroids with another immunomodulator treatment was necessary for all, because of frequent relapses. Methotrexate was effective in one case for the muscular and the respiratory signs, plasma exchanges with azathioprine in two cases, and intravenous immunoglobulin in one patient with acute lung injury.
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PMID:[Course of interstitial lung diseases associated with inflammatory myopathies. Apropos of 5 cases]. 913 92

The goal of treatment for juvenile rheumatoid arthritis (JRA) and other pediatric rheumatic disorders is to minimize joint destruction, pain, and deformity and to maximize all aspects of growth and development. Oral and injectable methotrexate are now often given early in the treatment of JRA, childhood dermatomyositis, difficult-to-control arthritis in the pediatric spondyloarthropathies, SLE, sarcoidosis, several of the vasculopathies, and idiopathic iritis. Weekly low-dose MTX has become a mainstay of long-term improved control of these disorders, and is associated with strikingly few documented long-term side effects. Dosages, pharmacology, side effects, efficacy, and treatment strategies are discussed. Although formal studies are lacking, MTX for the pediatric rheumatic disorders seems to be associated with less frequent physician visits, lower total costs, improved function, and fewer late reconstructive surgeries.
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PMID:Methotrexate in the treatment of juvenile rheumatoid arthritis and other pediatric rheumatoid and nonrheumatic disorders. 936 Nov 57

Methotrexate (MTX) nodulosis in patients with rheumatoid arthritis treated with MTX has become a well recognized phenomenon. It has not been described in patients receiving MTX for treatment of other diseases, e.g., dermatological or malignant diseases. Recently, MTX nodulosis was described in a patient with psoriasis and arthritis. The pathophysiology and treatment of MTX nodulosis are yet unsettled. We experienced a case with dermatomyositis who developed multiple subcutaneous nodules after treatment with MTX. In our patient, numerous, small, symmetrically distributed, grouped subcutaneous nodules were seen on a V-shaped area of the anterior chest, both axillary areas, and the medial sides of both upper arms and thighs. We histopathologically examined a nodule and started treatment with hydroxychloroquine after discontinuation of MTX. The histopathology revealed septal panniculitis, and the nodules have been under regression. MTX nodulosis may be a drug-specific phenomenon. Discontinuation of MTX and replacement with hydroxychloroquine are recommended in severe cases. Awareness of this entity is important for diagnosing and treating cases of multiple nodules developing after the administration of MTX, which is widely used for many dermatological diseases.
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PMID:Methotrexate nodulosis. 1045 88

Patients with polymyositis or dermatomyositis should be treated with prednisone (approximately 1 mg/kg/d) for an initial period of 4 to 6 weeks. Once improvement occurs, the dose should be tapered and converted to an alternate-day regimen, which should be continued for at least 12 months. Methotrexate or azathioprine should be administered concomitantly to patients in whom there is inadequate control. The early introduction of one of these drugs allows more rapid reduction in the dose of prednisone and helps to avert serious side effects. Intravenous immunoglobulin therapy is indicated for patients who have immunodeficiency, who are unable to tolerate immu-nosuppressive drugs, whose conditions are deteriorating, or who have severe relapses. Cyclosporine or cyclophosphamide may be effective for resistant disease. Patients with inclusion body myositis should undergo a 3- to 6-month trial of prednisone, alone or in combination with methotrexate or azathioprine. Maintenance doses of these drugs should be continued if the patient's condition improves or stabilizes.
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PMID:Inflammatory Myopathy. 1109 14

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

It is well known that a certain percentage of patients with polymyositis and dermatomyositis (PM/DM) is corticosteroid resistant. Established and novel approaches to steroid-resistant PM/DM are discussed in this review. Methotrexate (MTX) is a first-line treatment in the case that steroid therapy fails. Azathioprine and cyclophosphamide also fall into this category. Cyclosporine, a specific inhibitor of calcineurin, has been reported almost as effective as MTX. Tacrolimus, also a calcineurin inhibitor, and mycophenolate mofetil could be additional alternatives for the treatment. Several clinical trials have demonstrated that high-dose intravenous immunoglobulin is promising. Recently favorable data have been published using intravenous high-dose pulse cyclophosphamide or cyclosporine for the poorly prognostic interstitial pneumonitis or pulmonary fibrosis accompanied with PM/DM.
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PMID:[Treatment of steroid-resistant polymyositis and dermatomyositis]. 1167 54


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