Bcl-2, p53 and c-myc expression in juvenile dermatomyositis.

OBJECTIVE To investigate p53, bcl-2 and c-myc expression in muscle biopsies from children affected with juvenile dermatomyositis (JDM) and to verify a possible dysregulation of programmed cell death in this autoimmune disease. METHODS Ten muscle biopsies of children affected with JDM were formalin fixed and paraffin embedded. After haematoxylin and eosin staining, immunohistochemistry was performed employing monoclonal antibodies, anti-p53, anti-bcl-2 and anti-myc. Two normal muscle biopsies were studied as controls. RESULTS In the biopsies of JDM, two different patterns of myofibers damage were observed: the first, with zones characterised by necrosis; and the second, with zones where an apoptotic process was dominant. Immunoreactivity for bcl-2 was positive in 8 out of 10 biopsies. P53 and c-myc expression were not present in any case. No relationship between the degree of bcl-2 immunostaining and the disease course or outcome was observed. CONCLUSIONS The over-expression of bcl-2 protein in JDM may suggest a dysregulation of apoptosis in myofibers. Further studies are required in order to better understand the role of our data in the pathogenetic pathways of the disease.

[1]  F. Sell-Salazar.  [Juvenile dermatomyositis]. , 2002, Revista de neurologia.

[2]  D. Woods,et al.  Regulation of p53 function. , 2001, Experimental cell research.

[3]  I. Ferrer,et al.  Bcl-2 and Bax protein expression in human myopathies , 1999, Journal of the Neurological Sciences.

[4]  S. Schwartz,et al.  Death by any other name. , 1995, The American journal of pathology.

[5]  D. Isenberg,et al.  Bcl-2 expression is unaltered in unfractionated peripheral blood mononuclear cells in patients with systemic lupus erythematosus. , 1995, British journal of rheumatology.

[6]  K. Gatter,et al.  What is the value of bcl‐2 protein detection for histopathologists? , 1995, Histopathology.

[7]  L. Donehower,et al.  Evidence that p53 and bcl-2 are regulators of a common cell death pathway important for in vivo lymphomagenesis. , 1994, Oncogene.

[8]  N. Talal.  Oncogenes, autogenes, and rheumatic diseases. , 1994, Arthritis and rheumatism.

[9]  J. Mountz,et al.  Autoimmune disease. A problem of defective apoptosis. , 1994, Arthritis and rheumatism.

[10]  U. Jaeger,et al.  High levels of bcl-2 protein in circulating T lymphocytes, but not B lymphocytes, of patients with systemic lupus erythematosus. , 1994, Arthritis and rheumatism.

[11]  H. Hermeking,et al.  Mediation of c-Myc-induced apoptosis by p53. , 1994, Science.

[12]  R. Poulsom,et al.  BCL‐2 expression in adult and embryonic non‐haematopoietic tissues , 1993, The Journal of pathology.

[13]  G. Deng,et al.  Suppression of apoptosis in a cytotoxic T-cell line by interleukin 2-mediated gene transcription and deregulated expression of the protooncogene bcl-2. , 1993, Proceedings of the National Academy of Sciences of the United States of America.

[14]  L. Tucker,et al.  Methotrexate treatment of recalcitrant childhood dermatomyositis. , 1992, Arthritis and rheumatism.

[15]  Y. Tsujimoto,et al.  Analysis of the structure, transcripts, and protein products of bcl-2, the gene involved in human follicular lymphoma. , 1986, Proceedings of the National Academy of Sciences of the United States of America.

[16]  A. Masi,et al.  Polymyositis and dermatomyositis in children: an epidemiologic and clinical comparative analysis. , 1982, The Journal of rheumatology.

[17]  L. Pachman,et al.  Juvenile dermatomyositis: a clinical and immunologic study. , 1980, The Journal of pediatrics.

[18]  A. Bohan,et al.  Polymyositis and dermatomyositis (second of two parts). , 1975 .