Avian scleroderma: evidence for qualitative and quantitative T cell defects

J Autoimmun. 1992 Jun;5(3):261-76. doi: 10.1016/0896-8411(92)90142-d.


T cell activation is dependent upon calcium influx and protein kinase C activation, with subsequent lymphocyte proliferation dependent upon IL-2. Abnormalities in T cell proliferation, including abnormal calcium influx and defective protein kinase C activation, have been identified in aged mice and humans and many autoimmune diseases including diabetes, lupus and scleroderma. Since UCD line 200 chickens, which spontaneously develop a scleroderma-like disease, have both thymic defects and a diminished peripheral blood lymphocyte response to IL-2, we have further investigated T cell function in these birds. Interestingly, line 200 T cells respond poorly in vitro to a variety of diversely acting T cell mitogens including concanavalin A, phytohemagglutinin and anti-chicken CD3 monoclonal antibody. Moreover, they do not respond well even to phorbol myristate acetate in conjunction with ionomycin. Addition of exogenous IL-2-containing supernatant concurrently with mitogenic stimulation also had no significant effect. Analysis of intracellular free calcium demonstrated that the lymphocytes from diseased birds had a reduced influx of calcium (or release for intracellular stores) following stimulation. These data clearly reflect a unique defect in T cell activation associated with avian scleroderma. Analysis of chicken CD3, CD4 and CD8 expression revealed a 39% decrease in peripheral blood CD4+ cells in scleroderma birds, although this decrease was not sufficient to explain the 80-90% decrease observed in proliferation assays and calcium influx. Our data support the hypothesis that avian scleroderma is mediated via abnormal function of lymphocyte co-stimulatory molecules or intracellular calcium regulators.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Antigens, Differentiation, T-Lymphocyte / analysis
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / veterinary*
  • Calcium / metabolism
  • Chickens / genetics
  • Chickens / immunology*
  • Disease Models, Animal
  • Genes, MHC Class I
  • Leukocyte Count
  • Lymphocyte Activation / drug effects
  • Mitogens
  • Poultry Diseases / genetics
  • Poultry Diseases / immunology*
  • Scleroderma, Systemic / genetics
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / veterinary*
  • T-Lymphocyte Subsets* / drug effects


  • Antigens, Differentiation, T-Lymphocyte
  • Mitogens
  • Calcium