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Amyloidosis: An Unusual Cause of Intestinal Pseudo-Obstruction
Accepted Manuscript Amyloidosis: an unusual cause of intestinal pseudo-obstruction Jie Wu, Yongjun Wang, Chunlian Wang
PII: DOI: Reference:
S1542-3565(17)30820-0 10.1016/j.cgh.2017.07.002 YJCGH 55339
To appear in: Clinical Gastroenterology and Hepatology Accepted Date: 7 July 2017 Please cite this article as: Wu J, Wang Y, Wang C, Amyloidosis: an unusual cause of intestinal pseudoobstruction, Clinical Gastroenterology and Hepatology (2017), doi: 10.1016/j.cgh.2017.07.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Amyloidosis: an unusual cause of intestinal pseudo-obstruction Jie Wu, Yongjun Wang, Chunlian Wang Department of Gastroenterology, The Second Xiangya Hospital of Central South
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University, Changsha, Hunan, China
Corresponding author: Chunlian Wang, MD, Department of Gastroenterology, The
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Second Xiangya Hospital of Central South University, 139 Middle Renmin Road,
Email: [email protected] Tel: +86 13973161116 Fax: 0731-85533525
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Changsha, Hunan, 410011, China
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Author Contributions: Jie Wu, drafting of the manuscript; Yongjun Wang, clinical care of the patient; Chunlian Wang, study supervision.
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Conflicts of interest: None.
ACCEPTED MANUSCRIPT A 63-year-old woman presented with intermittent abdominal pain and vomiting over one month. The pain was located in the periumbilical area, and the vomiting was associated with postprandial occurrence. The symptoms resolved spontaneously, and
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her medical history was insignificant. Physical examination indicated a soft abdomen, and no masses were palpable. An occult blood test of the stool was positive. Complete blood count, rheumatologic testing, tumor biomarkers and erythrocyte sedimentation
SC
rate (ESR) were all within the normal range. A radiograph of abdomen performed at
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symptom onset showed dilatation of the small bowel loops (Figure A).
Esophagogastroduodenoscopy (EGD) revealed no abnormities of the esophagus or stomach, but nodules and diffuse erosions with mucosal fragility could be seen from the duodenal bulb to the horizontal part (Figure B, left). Colonoscopy with intubation
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of the terminal ileum to 15 cm identified normal colonic mucosa, but pale mucosa with a granular appearance was present in the terminal ileum (Figure B, right). Computed tomographic enterography (CTE) demonstrated diffuse wall thickening of
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the small bowel (Figure C).
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Biopsies of the lesions in the bulb-descending junction and horizontal part of the duodenum and in the terminal ileum showed eosinophilic depositions in the lamina propria (Figure D, left, from the horizontal part of the duodenum). Congo red staining further confirmed amyloidosis (Figure D, right). A bone marrow biopsy was performed to exclude multiple myeloma, and the results revealed an elevated proportion of plasma cells of 19% and no detectable neoplastic plasma cells. Immunofixation showed the presence of IgA lambda type M protein in the serum. No
ACCEPTED MANUSCRIPT other sites of amyloid deposition were found in the body. Thus, primary AL amyloidosis of the small intestine was diagnosed. The patient’s symptoms were relieved after conservative treatment, and the patient was discharged at her request.
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Amyloidosis is characterized by the extracellular deposition of amyloid protein. AL
amyloidosis (or primary amyloidosis) and AA amyloidosis are the two most common
SC
forms. AL amyloidosis is associated with plasma cell dyscrasia, in which monoclonal light chains in serum or urine can be detected, whereas AA amyloidosis with serum
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amyloid A protein depositions is often secondary to chronic inflammation, infection and neoplasms.1 AL amyloidosis commonly involves multiple organs such as the heart, kidney, liver, and GI tract. Amyloidosis confined to the GI tract is rare. Small bowel involvement is the greatest when the GI tract is affected by amyloid. The
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associated symptoms may include diarrhea, hemorrhage, malabsorption, obstruction, perforation, and even pseudo-obstruction. Endoscopic findings include mucosal
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fragility, diffuse lesions such as ulcers, erosions, granular appearance, polyps, etc.2 In the present case, the amyloidosis was confined to the small intestine and had caused
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extensive involvement of the small bowel. The patient presented with the rare manifestation of intestinal pseudo-obstruction, which could have arisen from impaired intestinal motility caused by the amyloid depositions.3 This case demonstrates the importance of using endoscopy to evaluate the presence of amyloidosis and amyloidosis as a potential cause of intestinal pseudo-obstruction.
ACCEPTED MANUSCRIPT
References 1.
Picken MM. New insights into systemic amyloidosis: the importance of diagnosis of specific
2.
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type. Current opinion in nephrology and hypertension. 2007;16(3):196-203. Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. The American journal of
gastroenterology. 2008;103(3):776-87.
Deguchi M, Shiraki K, Okano H, et al. Primary localized amyloidosis of the small intestine
SC
3.
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presenting as an intestinal pseudo-obstruction: report of a case. Surgery today.
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2001;31(12):1091-3.
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SC
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PII: DOI: Reference:
S1542-3565(17)30820-0 10.1016/j.cgh.2017.07.002 YJCGH 55339
To appear in: Clinical Gastroenterology and Hepatology Accepted Date: 7 July 2017 Please cite this article as: Wu J, Wang Y, Wang C, Amyloidosis: an unusual cause of intestinal pseudoobstruction, Clinical Gastroenterology and Hepatology (2017), doi: 10.1016/j.cgh.2017.07.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Amyloidosis: an unusual cause of intestinal pseudo-obstruction Jie Wu, Yongjun Wang, Chunlian Wang Department of Gastroenterology, The Second Xiangya Hospital of Central South
RI PT
University, Changsha, Hunan, China
Corresponding author: Chunlian Wang, MD, Department of Gastroenterology, The
SC
Second Xiangya Hospital of Central South University, 139 Middle Renmin Road,
Email: [email protected] Tel: +86 13973161116 Fax: 0731-85533525
M AN U
Changsha, Hunan, 410011, China
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Author Contributions: Jie Wu, drafting of the manuscript; Yongjun Wang, clinical care of the patient; Chunlian Wang, study supervision.
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Conflicts of interest: None.
ACCEPTED MANUSCRIPT A 63-year-old woman presented with intermittent abdominal pain and vomiting over one month. The pain was located in the periumbilical area, and the vomiting was associated with postprandial occurrence. The symptoms resolved spontaneously, and
RI PT
her medical history was insignificant. Physical examination indicated a soft abdomen, and no masses were palpable. An occult blood test of the stool was positive. Complete blood count, rheumatologic testing, tumor biomarkers and erythrocyte sedimentation
SC
rate (ESR) were all within the normal range. A radiograph of abdomen performed at
M AN U
symptom onset showed dilatation of the small bowel loops (Figure A).
Esophagogastroduodenoscopy (EGD) revealed no abnormities of the esophagus or stomach, but nodules and diffuse erosions with mucosal fragility could be seen from the duodenal bulb to the horizontal part (Figure B, left). Colonoscopy with intubation
TE D
of the terminal ileum to 15 cm identified normal colonic mucosa, but pale mucosa with a granular appearance was present in the terminal ileum (Figure B, right). Computed tomographic enterography (CTE) demonstrated diffuse wall thickening of
EP
the small bowel (Figure C).
AC C
Biopsies of the lesions in the bulb-descending junction and horizontal part of the duodenum and in the terminal ileum showed eosinophilic depositions in the lamina propria (Figure D, left, from the horizontal part of the duodenum). Congo red staining further confirmed amyloidosis (Figure D, right). A bone marrow biopsy was performed to exclude multiple myeloma, and the results revealed an elevated proportion of plasma cells of 19% and no detectable neoplastic plasma cells. Immunofixation showed the presence of IgA lambda type M protein in the serum. No
ACCEPTED MANUSCRIPT other sites of amyloid deposition were found in the body. Thus, primary AL amyloidosis of the small intestine was diagnosed. The patient’s symptoms were relieved after conservative treatment, and the patient was discharged at her request.
RI PT
Amyloidosis is characterized by the extracellular deposition of amyloid protein. AL
amyloidosis (or primary amyloidosis) and AA amyloidosis are the two most common
SC
forms. AL amyloidosis is associated with plasma cell dyscrasia, in which monoclonal light chains in serum or urine can be detected, whereas AA amyloidosis with serum
M AN U
amyloid A protein depositions is often secondary to chronic inflammation, infection and neoplasms.1 AL amyloidosis commonly involves multiple organs such as the heart, kidney, liver, and GI tract. Amyloidosis confined to the GI tract is rare. Small bowel involvement is the greatest when the GI tract is affected by amyloid. The
TE D
associated symptoms may include diarrhea, hemorrhage, malabsorption, obstruction, perforation, and even pseudo-obstruction. Endoscopic findings include mucosal
EP
fragility, diffuse lesions such as ulcers, erosions, granular appearance, polyps, etc.2 In the present case, the amyloidosis was confined to the small intestine and had caused
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extensive involvement of the small bowel. The patient presented with the rare manifestation of intestinal pseudo-obstruction, which could have arisen from impaired intestinal motility caused by the amyloid depositions.3 This case demonstrates the importance of using endoscopy to evaluate the presence of amyloidosis and amyloidosis as a potential cause of intestinal pseudo-obstruction.
ACCEPTED MANUSCRIPT
References 1.
Picken MM. New insights into systemic amyloidosis: the importance of diagnosis of specific
2.
RI PT
type. Current opinion in nephrology and hypertension. 2007;16(3):196-203. Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. The American journal of
gastroenterology. 2008;103(3):776-87.
Deguchi M, Shiraki K, Okano H, et al. Primary localized amyloidosis of the small intestine
SC
3.
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presenting as an intestinal pseudo-obstruction: report of a case. Surgery today.
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2001;31(12):1091-3.
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SC
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