YOU ARE NOW CONNECTED TO THE TOXLINE (1981 FORWARD, NON-ROYALTY) FILE. ==DERMATOMYOSITIS== 6 AUTHOR Plotz PH AUTHOR Dalakas M AUTHOR Leff RL AUTHOR Love LA AUTHOR Miller FW AUTHOR Cronin ME TITLE Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders. SOURCE Ann Intern Med; VOL 111, ISS 2, 1989, P143-57 (REF: 100) ABSTRACT Idiopathic inflammatory myopathy, a category encompassing polymyositis, dermatomyositis, and a number of other disorders, is very uncommon, but has been the focus of intense study in the Arthritis and Rheumatism Branch of the National Institute of Arthritis and Musculoskeletal and Skin Diseases for the past several years. We describe the clinical picture, stressing the need for biopsy to ensure correct diagnosis. It is especially important to recognize the treatment-resistant variant, inclusion body myositis. The extraskeletal manifestations, particularly the cardiopulmonary, oropharyngeal, gastrointestinal, and endocrine involvement, are described. The cardiopulmonary involvement, especially interstitial lung disease, arrhythmias, and cardiac failure, may dominate the clinical picture. The known causes are varied, and include drugs, toxins, and some infectious agents, however, in most cases a cause cannot yet be identified. Circumstantial evidence suggests that picornaviruses may initiate some cases in humans, and a very similar disease in mice caused by a picornavirus is actively under study. Studies of autoantibodies and cellular immune function support a central role for disordered immunity in the pathogenesis. The myositis-specific autoantibodies, especially those directed at certain enzymes important in protein synthesis (the aminoacyl-transfer RNA synthetases), are found in a clinically distinct subset of patients. Although most patients respond initially to corticosteroids, cytotoxic drugs are sometimes added when steroid toxicity or refractoriness develops. We describe several newer therapies under study for such cases and outline future directions in research. 2 AUTHOR Lueck CJ AUTHOR Trend P AUTHOR Swash M TITLE Cyclosporin in the management of polymyositis and dermatomyositis. SOURCE J Neurol Neurosurg Psychiatry; VOL 54, ISS 11, 1991, P1007-8 ABSTRACT Three patients with polymyositis refractory to conventional steroid and immunosuppressive treatment, but responsive to cyclosporin A, are described. In a fourth patient cyclosporin A was used as a first line drug in combination with steroids in the treatment of life-threatening dermatomyositis. Cyclosporin A in the management of polymyositis/dermatomyositis requires formal assessment of its costs and benefits compared with conventional treatments. 1 AUTHOR Chwalinska-Sadowska H AUTHOR Maldykowa H TITLE Polymyositis-dermatomyositis:25 years of follow-up of 50 patients disease course, treatment, prognostic factors. SOURCE Mater Med Pol; VOL 22, ISS 3, 1990, P213-8 (REF: 14) ABSTRACT 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. 4 AUTHOR Ramirez G AUTHOR Asherson RA AUTHOR Khamashta MA AUTHOR Cervera R AUTHOR D'Cruz D AUTHOR Hughes GR TITLE Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment. SOURCE Semin Arthritis Rheum; VOL 20, ISS 2, 1990, P114-20 ABSTRACT 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. 6 AUTHOR Plantin P AUTHOR Saraux A AUTHOR Guillet G TITLE [Methotrexate in dermatology: current aspects] SOURCE Ann Dermatol Venereol 1989;116(2):109-15 ABSTRACT The prescription of methotrexate (MTX) in dermatology is nowadays restricted for two main reasons: the toxicity of the drug and the availability of less toxic treatments (phototherapy and etretinate) in its main indication: psoriasis. However, MTX remains very useful in severe psoriasis, to some extent, in various dermatological diseases, such as dermatomyositis. A perfect knowledge of its adverse effects (slight on the gastrointestinal tract and bone marrow, much more serious on the liver and lung) allows a reasonable use of the drug but cannot avoid the unpredictable hypersensitivity pneumopathy.