Chronic disease of the liver associated with systemic scleroderma

Summary

Of 727 patients with systemic scleroderma, 8 had evidence of serious hepatic disease. This low incidence hardly suggests a relationship between the diffuse cutaneous sclerotic process and the chronic hepatic disease, although the association should always be looked for. It is not possible to exclude hepatitis and malnutrition as causative factors of the hepatic disease, although the histologic appearance did not suggest the latter. The hepatic disease usually developed insidiously after years of involvement by the sclerotic process. Similar disease of the liver previously noted in other related diffuse processes, such as lupus erythematosus, and the overlapping of these diffuse processes, particularly as noted in the first case, are further suggestive of a possible etiologic relationship. Special studies of the ascitic fluid in one instance presented some direct evidence to support the concept that hepatic involvement was primary and not secondary to other causes such as cardiac involvement in scleroderma. Finally, it should be emphasized again that postnecrotic cirrhosis is a morphologic and not an etiologic diagnosis and may result from many diverse causes. Careful study of the liver and its function in future instances of diffuse systemic disease such as scleroderma and lupus erythematosus may shed further light on this relationship.