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Gastroenterology & Hepatology, 1103 Clinical Sciences

Abstract:

Objective: Gastrointestinal involvement in amyloidosis is very frequent. Clinical manifestations and related findings are variable. Steatorrhoea and malabsorption are common findings in familial forms of amyloidosis but less common in AL (light chain protein) and AA amyloidosis (A-protein). The aetiopathogenesis of the steatorrhoea and malabsorption is unclear. The importance of neuromuscular amyloid deposits has been shown in familial amyloidosis. In order to study the malabsorption and relative involvement of the mucosa and neuromuscular components of the bowel wall and pancreas we reviewed in depth the biopsies and medical records of two patients with small bowel amyloidosis. Design: One case each of AL and AA amyloidosis is described with the diagnostic absorption tests and the light microscopic morphological findings. Results: The absorption tests suggest malabsorption as a consequence of bacterial overgrowth which could be caused by a motility disturbance in both cases. Morphological examination reveals amyloid deposition around both the extrinsic and intrinsic neural structures in the patient with AA amyloidosis. Conclusions: The basic mechanism of the malabsorption in small bowel amyloidosis therefore could be a neuropathy, at least in a number of cases. These findings are discussed in the light of a concommitant diabetes mellitus in the patient with AL amyloidosis and the literature findings in general.