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. Goodpasture's disease is characterized by rapidly  progressive glomerulonephritis and pulmonary hemorrhage, in association with  circulating and deposited anti-glomerular basement membrane antibodies that  recognize the α3 chain of type IV collagen [α3(IV)NC1] (known as  the Goodpasture antigen). Unlike many other autoimmune diseases, recurrences  are rare. In experimental models and human studies, both humoral and cellular  mechanisms have been demonstrated to be involved in disease pathogenesis.  However, there are few data on the characteristics of the autoreactive T cells  or the mechanisms of tolerance to the autoantigen in human patients. It was  demonstrated, using immunohistochemical analyses and reverse  transcription-PCR, that the Goodpasture antigen is expressed in normal human  thymus. Using limiting dilution analyses, the frequencies of circulating  autoreactive T cells in patients and control subjects were assessed. During  acute disease, there were increased frequencies of CD4 +  T cells  reactive with α3(IV)NC1 (ranging from 1:6300 to 1:65,000), which  decreased with time. There was a significant difference between patients  during their acute disease phase and control subjects with respect to the  frequency index for α3(IV)NC1-specific CD4 +  T cells  ( P  &lt; 0.05, Mann Whitney  U  test). The decrease in  autoreactive CD4 +  T-cell numbers during recovery may be the reason  why recurrences are infrequent and may explain the loss of pathogenic  autoantibodies with time, because of a lack of T-cell help.

In Goodpasture's Disease, CD4+ T Cells Escape Thymic Deletion and Are Reactive with the Autoantigen α3(IV)NC1