Splenic hypoperfusion as a sign of systemic amyloidosis.

BACKGROUND: The spleen is frequently involved in systemic amyloidosis; however, the computed tomographic (CT) or magnetic resonance (MR) pattern of splenic amyloidosis is not sufficiently described in the literature. This study evaluated the contrast-enhanced CT and MR findings of the spleen in patients with systemic amyloidosis. METHODS: Data were extracted by reviewing pathology and radiology department records of the teaching hospital of Naples over 10 years, from 1 January 1993 to 31 December 2002. Thirty-three patients with amyloidosis were identified, 10 of whom had a CT scan and two of whom had an MR study. The population-based study was composed of 12 patients with histologically proved amyloidosis who underwent contrast-enhanced CT or MR scan of the abdomen. The spleen and liver were evaluated for organ size and perfusion. RESULTS: The spleen was hypoperfused in nine of 12 patients. Mild splenomegaly was present in only one case. Hepatomegaly was associated with markedly acute left lobe margin in nine patients and with rounded anterior profile of segments 3 and 4 in four patients. Moreover, a large area of low attenuation with indefinite geographic margins involving the right hepatic lobe was observed in three patients. CONCLUSION: The finding of splenic hypoperfusion may be a marker of systemic amyloidosis, which represents a useful clue when clinical findings fail to suggest the proper diagnosis.

Selective reduction of intestinal trefoil factor in untreated coeliac disease.

The trefoil factor family (TFF) encompasses small peptides of which intestinal trefoil factor (ITF) is expressed specifically in goblet cells of the small and large intestine. Previous studies have shown that ITF plays an important role in mucosal protection and repair. Coeliac disease represents a model of immune-mediated small intestinal inflammation and damage, with recovery on gluten-free diet. The aim of this study was to investigate the expression of ITF in the distal duodenal mucosa of subjects with coeliac disease, before and after treatment with a gluten-free diet. Expression of ITF and mucin in the distal duodenal biopsies from treated (n = 11) and untreated (n = 9) coeliac subjects and controls (n = 8) was investigated by immunohistochemistry and semiquantitative PCR. In untreated coeliac disease, there was reduction of ITF immunoreactivity in goblet cells but mucin expression was preserved. Mucosal recovery on gluten-free diet was associated with increased ITF immunoreactivity in goblet cells. There was also reduction in the expression of ITF transcripts, relative to MUC2 mRNA, in untreated coeliac duodenal samples, with recovery on gluten-free diet. Our study suggests that there is a selective reduction in the expression of the ITF gene in untreated coeliac disease. Recovery of ITF expression on a gluten-free diet suggests that the mucosal immune system regulates goblet cell differentiation and ITF expression in the human intestinal mucosa.