Liver involvement in HHT is characterized by a high prevalence of FNH and a large spectrum of vascular lesions such as intrahepatic shunts, nodular regenerative hyperplasia, sinusoidal dilatation and ischaemic cholangiopathy that may coexist simultaneously in the same patient.

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The secretion of large volumes of fluid into cysts and changes in the structure and mobility of the cilia of the renal tubular epithelium can lead to nephromegaly. This in turn often causes a deterioration of kidney function and arterial hypertension. In recent clinical studies, somatostatin analogues have demonstrated efficacy in isolated polycystic liver disease and, to a lesser extent, in polycystic kidney disease. Since the publication of these clinical studies, several patients have been referred to us for somatostatin analogue treatment. Here, we report our experience with 6 patients who were treated with lanreotide autogel 120 mg every 4 weeks over 6, 12 or 18 months and were longitudinally followed using CT scans without contrast agents, to evaluate the total bilateral kidney volume. We observed a mean decrease in volume of 4%, with mild to moderate side effects.

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