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DOI: 10.1055/s-0030-1256420
© Georg Thieme Verlag KG Stuttgart · New York
Primary gastroduodenal amyloidosis
Henrik ThorlaciusMD
Department of Surgery and Clinical Sciences
Skåne
University Hospital
Lund University
S-205 02 Malmö
Sweden
Fax: +46-40-336207
Email: henrik.thorlacius@med.lu.se
Publication History
Publication Date:
13 September 2011 (online)
Amyloidosis is characterized by tissue deposition of insoluble fibrillar proteins in various organs [1]. In humans, more than 23 different and unrelated proteins are known to form amyloid fibrils [2]. Amyloidosis is divided into primary (i. e. idiopathic) and secondary amyloidosis (i. e. associated with chronic inflammatory conditions, and infectious and neoplastic disorders) [1]. Primary amyloidosis is extremely rare in the gastrointestinal tract. Fewer than 1 % of patients with primary amyloidosis in the gastrointestinal tract have any symptoms [3]. We report a case of primary gastroduodenal amyloidosis in which endoscopic ultrasound (EUS) was instrumental in the work-up.
A 76-year-old man presented with a history of fatigue, dyspepsia, and anemia. An upper-gastrointestinal endoscopy revealed prominent gastric folds and gastropathy ([Fig. 1 a]).
Gastric biopsies showed only signs of unspecific inflammation. The patient developed diarrhea and weight loss. Abdominal computed tomography (CT) showed unspecific gastric wall thickening. The initial suspicion was a malignant disease such as scirrhous carcinoma, which led to an EUS referral. EUS revealed gastric wall thickening ([Fig. 1 b]) and a complete lack of normal sonographic layers in the stomach wall, suggestive of an infiltrative disease ([Fig. 1 b]), but no sign of malignancy. Another upper-gastrointestinal endoscopy was undertaken with new biopsies from the stomach and bulbus duodeni. Congo red staining revealed amorphous eosinophilic infiltrates ([Fig. 2 a]) and green birefringence under polarized light ([Fig. 2 b]), which is diagnostic for amyloidosis [1].
It may be suggested that tissue deposition of insoluble proteins causes the sonographic disappearance of normal gastric wall layers in amyloidosis. This patient had no systemic disease such as a chronic immune disease or neoplastic disorder, which are the most common causes of secondary amyloidosis. Thus this patient had a primary amyloidosis in the gastroduodenum. In conclusion, this case highlights the use of EUS in the work-up of patients with suspected amyloidosis in the stomach and duodenum.
Endoscopy_UCTN_Code_CCL_1AB_2AD_3AD
Competing interests: None
#References
- 1 Ebert E C, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol. 2008; 103 776-787
- 2 Buxbaum J N. The systemic amyloidoses. Curr Opin Rheumatol. 2004; 16 67-75
- 3 Menke D M, Kyle R A, Fleming C R et al. Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis. Mayo Clin Proc. 1993; 68 763-767
Henrik ThorlaciusMD
Department of Surgery and Clinical Sciences
Skåne
University Hospital
Lund University
S-205 02 Malmö
Sweden
Fax: +46-40-336207
Email: henrik.thorlacius@med.lu.se
References
- 1 Ebert E C, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol. 2008; 103 776-787
- 2 Buxbaum J N. The systemic amyloidoses. Curr Opin Rheumatol. 2004; 16 67-75
- 3 Menke D M, Kyle R A, Fleming C R et al. Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis. Mayo Clin Proc. 1993; 68 763-767
Henrik ThorlaciusMD
Department of Surgery and Clinical Sciences
Skåne
University Hospital
Lund University
S-205 02 Malmö
Sweden
Fax: +46-40-336207
Email: henrik.thorlacius@med.lu.se