BwR
25MEDICAL JOURNAL abnormal in 8 out of 13 patients. However, the bromsulphthalein excretion and the percentage of esterified serum cholesterol were normal. Since these two tests are more specific, the possibility of misinterpretation of deranged protein metabolism as evidence of liver disease rather than as an integral feature of systemic scleroderma needs no emphasis.The hepatic fibrosis observed at operation in our first patient defies ready explanation, since there was no evidence of malnutrition, previous hepatic or biliary disease, generalized passive venous congestion, small intestinal defect, or ulcerative colitis. Though not subject to proof, it may be a direct manifestation of the viscero-sclerodermatous process. Massimo (1954) Eventual hepatic fibrosis is a recognized sequel to congestive hepatomegaly from cardiac and pulmonary scleroderma and might also be expected to occur in some patients with sclerodermatous haemorrhagic colitis, especially if there were concomitant steatorrhoea as exemplified in our second patient. Her " silver stool " was regarded as a product of these abnormalities. It is, however, equally admitted that the hepatic fibrosis and its complications in our two patients might be independent of the sclerodermatous process.Finally, we suggest that the possibility of the presence of oesophageal varices, as witnessed in both our patients, should be entertained during the standard radiological studies of visceral scleroderma.
SummaryTwo illustrative cases, both in women, of scleroderma and hepatic fibrosis are fully reported. In each patient the salient feature was portal hypertension, including oesophageal varices, which led to recurrent haematemesis, an aspect apparently not previously described.The first patient survived a palliative subcardiac portoazygos disconnexion and remained well five years later, with no subsequent haematemesis. The second patient, whose illness was further complicated by a combination of sclerodermatous haemorrhagic colitisandsteatorrhoea to produce the " silver stool " sign, was treated conservatively for repetitive haematemesis. After prolonged invalidism she died in coma, as a result of haemorrhage from an oesophageal varix, some 20 months after her first haematemesis.The scant literature on hepatic fibrosis in systemic scleroderma is reviewed and the causation of such hepatic lesions is briefly discussed in relation to the observations on our two patients.Our thanks are due to Mr. N. C. Tanner for permission ts refer to the operative details in Case 1; to Mr. K. Moore for similar authority in relation to Case