T-cell subsets in scleroderma patients

Systemic sclerosis (SSc), or scleroderma, is characterized by fibrosis of the skin and internal organs, vascular abnormalities and alterations of the immune response compartment. T cells are present in affected tissues, including the skin, early in the disease course, appear to be oligoclonal, indicating an antigen-driven process, and preferentially produce Th2 cytokines, including IL-4 and IL-13, which may directly favor excessive extracellular matrix deposition. Th1 cells may also be present. Initial determinations indicate that Th17 numbers are increased and T cells with regulatory functions are defective. Of interest, genetic association studies have preferentially identified polymorphisms of genes coding for molecules belonging to the immune system as risk factors for SSc development, further stressing the importance of immune dysregulation in SSc pathobiology. While current therapeutic approaches remain disappointing, we discuss the advance in our understanding of the immune abnormalities characteri...