Abstract

Severe idiopathic inflammatory myopathies (polymyositis, dermatomyositis, and related diseases) that respond poorly to corticosteroids are difficult to treat. We have undertaken three therapeutic trials for the treatment of such patients: a) a controlled double-blind trial of phasmapheresis and leukapheresis; b) a controlled, crossover trial of intravenous methotrexate with leucovorin rescue and combination of methotrexate and azathioprine; c) an open trial of i.v. cyclophosphamide (completed). The trial of apheresis has just been closed after 39 patients have completed it. Analysis of the results is underway. The trial of i.v. methotrexate and combination therapy continues, although patients with inclusion body myositis are no longer being entered because of poor results in the first 12 patients randomized.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Type
Intramural Research (Z01)
Project #
1Z01AR041076-04
Application #
3804556
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
4
Fiscal Year
1991
Total Cost
Indirect Cost

  Institution

Name
National Institute of Arthritis and Musculoskeletal and Skin Diseases
Department
Type
DUNS #
City
State
Country
United States
Zip Code

  Publications

Alger, Heather M; Raben, Nina; Pistilli, Emidio et al. (2011) The role of TRAIL in mediating autophagy in myositis skeletal muscle: a potential nonimmune mechanism of muscle damage. Arthritis Rheum 63:3448-57
Nagaraju, Kanneboyina; Rawat, Rashmi; Veszelovszky, Edina et al. (2008) Dysferlin deficiency enhances monocyte phagocytosis: a model for the inflammatory onset of limb-girdle muscular dystrophy 2B. Am J Pathol 172:774-85
O'Hanlon, Terrance P; Carrick, Danielle Mercatante; Targoff, Ira N et al. (2006) Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies. Medicine (Baltimore) 85:111-27
O'Hanlon, Terrance P; Carrick, Danielle Mercatante; Arnett, Frank C et al. (2005) Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1 and -DQA1 allelic profiles and motifs define clinicopathologic groups in caucasians. Medicine (Baltimore) 84:338-49
Sarkar, Kakali; Weinberg, Clarice R; Oddis, Chester V et al. (2005) Seasonal influence on the onset of idiopathic inflammatory myopathies in serologically defined groups. Arthritis Rheum 52:2433-8
Christopher-Stine, Lisa; Plotz, Paul H (2004) Adult inflammatory myopathies. Best Pract Res Clin Rheumatol 18:331-44
Plotz, P (2004) The place of autoimmunity in myositis. Autoimmun Rev 3 Suppl 1:S36
Isenberg, D A; Allen, E; Farewell, V et al. (2004) International consensus outcome measures for patients with idiopathic inflammatory myopathies. Development and initial validation of myositis activity and damage indices in patients with adult onset disease. Rheumatology (Oxford) 43:49-54
Miller, F W; Rider, L G; Plotz, P H et al. (2004) Polymyositis: an overdiagnosed entity. Neurology 63:402; author reply 403
Christopher-Stine, Lisa; Plotz, Paul H (2004) Myositis: an update on pathogenesis. Curr Opin Rheumatol 16:700-6