摘要：

Sjgren's syndrome occurred in 37 per cent, renal tubular acidosis in 32 per cent, pulmonary diffusion defects in 26 per cent and peripheral neuropathy in 10 per cent of patients with active chronic hepatitis, primary biliary cirrhosis or cryptogenic cirrhosis. The incidence of certain other conditions determined from clinical features alone was lower; these included arthropathy in 14 per cent, thyroid disorders in 10 per cent, skin lesions in 17 per cent and colitis in 5 per cent. In the complete series of 218 patients, 125 (57 per cent) had involvement of at least one organ other than the liver, such involvement being significantly more common in those with active chronic hepatitis (63 per cent of cases) and primary biliary cirrhosis (68 per cent) than in those with cryptogenic cirrhosis (38 per cent). In a number of patients, prednisone therapy was followed by both subjective and objective improvement in the features of the multisystem involvement. No correlation could be found between multisystem involvement and the presence of mitochondrial, smooth muscle or antinuclear antibodies in the serum or with the serum levels of immunoglobulins A (IgA), M (IgM) and G (IgG). Histologie examination of the various organs disclosed dense infiltration with small lymphocytes, suggesting that delayed hypersensitivity reactions were involved in the production of tissue damage. In support of this was the demonstration of cell-mediated reactivity in vitro to salivary or renal antigens in 42 per cent of the patients with Sjgren's syndrome and in 62 per cent of those with renal tubular acidosis, respectively. These findings, together with the frequency and similar pattern of multisystem involvement in the three conditions, suggest a common pathogenetic mechanism, and disordered cellular immune reactions directed primarily against the liver could affect other organs as a result of cross antigenicity.

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