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Chronic Diarrhea - Never Overlook Gastrointestinal Amyloidosis

Authors:
  • Prime west consortium/West Anaheim Medical Center
  • Southeast health
RESEARCH POSTER PRESENTATION DESIGN © 2022
www.PosterPresentations.com
Amyloidosis is an extremely rare disease
frequently diagnosed at an advanced stage,
resulting in significant morbidity and mortality.
Gastrointestinal amyloidosis can present with
unintentional weight loss, diarrhea, abdominal
pain, malabsorption, gastroesophageal reflux, and
GI bleeding. We present a case of chronic diarrhea
in a patient with gastrointestinal amyloidosis due to
long-standing chronic inflammation.
INTRODUCTION
CASE PRESENTATION
Gastroduodenoscopy revealed gastritis (Fig a). A colonoscopy revealed erythema and slightly raised mucosa
throughout the large bowel (Fig b). Histopathological examination of gastric and intestinal mucosa biopsies
showed amyloidosis (Fig c). Congo red stain of the gastric and intestinal mucosa revealed apple green
birefringence (fig d).
IMAGING AND HISTOPATHOLOGYDISCUSSION
Amyloidosis is an extremely rare disease
frequently missed or diagnosed at an advanced
stage, resulting in significant morbidity and
mortality. Amyloidosis develops when abnormal
protein fibrils deposit in the mucosa. It can present
with unintentional weight loss, diarrhea, abdominal
pain, malabsorption, gastroesophageal reflux, and
GI bleeding. Secondary AA amyloidosis can be a
complication of any long-term inflammatory
diseases like collagen diseases and infectious
disorders. However, as underlying conditions are
better managed, the prevalence of AA amyloidosis
is decreasing. Endoscopic appearance is subtle and
non-specified, even with extensive amyloid
deposition. Congo-red staining on tissue is the gold
standard for diagnosing amyloidosis. Treatment
should address the symptoms and underlying
conditions causing amyloid deposition. Chronic
diarrhea and protein-losing enteropathy caused by
gastrointestinal amyloid can be treated with long-
acting somatostatin analogs or octreotide and total
parenteral nutrition. Loperamide, opiates, and
empiric antibiotic treatment for small intestinal
bacterial overgrowth are other options for diarrhea
treatment. The prognosis is influenced by the
underlying disease that causes amyloid production.
CONCLUSION
Because of the non-specificity of symptoms that
mimic more common conditions, there is arisk of
diagnostic delay. Clinicians should thoroughly
investigate the gastrointestinal tract in patients
with refractory diarrhea and weight loss to rule
out gastrointestinal amyloidosis after other
diagnoses have been ruled out.
REFERENCES
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AA amyloidosis secondary to chronic pyelonephritis presenting with refractory diarrhea and severe hypoalbuminemia. Clinical journal of gastroenterology,14(6), 1642–1648.
https://doi.org/10.1007/s12328-021-01508-1
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A 74-year-old female with a past medical history of
Hepatitis C with cirrhosis, chronic recurrent
bronchiectasis, MAC complex colonization in the
lung, untreated latent tuberculosis, and systemic
lupus erythematosus presented with 7-10 episodes
of watery diarrhea worsened with food intake and
occasionally associated with hematochezia for past
four months. She was admitted twice for similar
complaints in the past three months. She has had a
20-pound weight loss in 4 months. Stool studies
were unremarkable. Gastroenterology was
consulted for chronic diarrhea and weight loss.
Gastroduodenoscopy revealed gastritis. A
colonoscopy revealed erythema and slightly raised
mucosa throughout the large bowel. The pathology
report from gastric and intestinal mucosa biopsies
showed amyloidosis. Serum protein electrophoresis
was negative, ruling out primary amyloidosis,
indicating likely AA amyloidosis from long-
standing inflammation. The patient was started on
antiemetics, loperamide, rifaximin, and parenteral
nutrition, with only a slight improvement in
symptoms.
1Department of Internal Medicine, West Anaheim Medical Center, Anaheim, California, US
2Department of Internal Medicine, Southeast Health, Dothan, Alabama, US
3Department of Gastroenterology, West Anaheim Medical Center, Anaheim, California, US
Yamini Katamreddy, M.D.1; Saikiran Mandyam, M.D.2; Thomas J Konturek M.D.3
CHRONIC DIARRHEA? NEVER OVERLOOK GASTROINTESTINAL AMYLOIDOSIS
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Article
Full-text available
Amyloidosis is a rare disease caused by extracellular deposits of insoluble fibrillar proteins in various organs and tissues. There are different forms of amyloidosis distinguished by the type of protein fibrils, by the sites of deposition and by associated conditions. Gastrointestinal involvement is common both in primary and secondary amyloidosis, while isolated gastrointestinal amyloidosis is rare. We describe a case of AL amyloidosis with a gastrointestinal involvement and restrictive cardiomiopathy. A 64 year old woman came to our attention with a history of chronic diarrhoea and weight loss, associated with dysphagia, dry mouth, xerophtalmia, chronic gastritis and depression. Clinical diagnosis has been difficult because of aspecificity of symptoms that mimed other more common diseases, like gastro-paresis, epigastric discomfort, gastric or duodenal ulcers, perforation, malabsorption, intestinal pseudo-obstruction. There is an important risk of misunderstanding and diagnostic delay. Indeed in this patient a diagnosis of irritable colon syndrome was erroneously established two years before admission in our hospital. Therefore gastrointestinal amyloidosis should be considered among differential diagnoses of chronic diarrhoea and weight loss when other more common diseases have been excluded.
Article
AA amyloidosis, previously known as secondary amyloidosis, has been associated with multiple chronic inflammatory conditions, including various autoimmune diseases and rarely chronic infection. Hereby, we present a case of AA amyloidosis secondary to chronic infection which initially presented with nausea and hematemesis. Endoscopic biopsies revealed diffuse AA amyloid deposition in the stomach, but not the esophagus. AA Amyloidosis presumably compromised gastric motility, promoted reflux related esophageal ulcers and erosions, and caused his cardiac and renal insufficiency. Therefore, endoscopic biopsies could be practical investigation to identify AA amyloidosis in the setting of chronic inflammatory diseases, especially with multi-organ involvement.
Isolated amyloidosis of the gastro-intestinal tract
  • S Smeets
  • F Dedeurwaerdere
  • E Thomaere
  • B Houthoofd
  • L D'hulst
  • P Wilmes
  • F Offner
  • S De Coninck
Smeets, S., Dedeurwaerdere, F., Thomaere, E., Houthoofd, B., D'Hulst, L., Wilmes, P., Offner, F., & De Coninck, S. (2022). Isolated amyloidosis of the gastro-intestinal tract. Acta gastroenterologica Belgica, 85(1), 80-84. https://doi.org/10.51821/85.1.8499
Gastric amyloidosis presenting with severe weight loss
  • S Biswas
  • J Iqbal
  • A Makin
Biswas, S., Iqbal, J., & Makin, A. (2010). Gastric amyloidosis presenting with severe weight loss. BMJ case reports, 2010, bcr0120102650. https://doi.org/10.1136/bcr.01.2010.2650
Secondary, AA, Amyloidosis. Rheumatic diseases clinics of North America
  • R Papa
  • H J Lachmann
Papa, R., & Lachmann, H. J. (2018). Secondary, AA, Amyloidosis. Rheumatic diseases clinics of North America, 44(4), 585-603. https://doi.org/10.1016/j.rdc.2018.06.004
Infections and AA amyloidosis: An overview
  • S Deshayes
  • A Aouba
  • G Grateau
  • S Georgin-Lavialle
Deshayes, S., Aouba, A., Grateau, G., & Georgin-Lavialle, S. (2021). Infections and AA amyloidosis: An overview. International journal of clinical practice, 75(6), e13966. https://doi.org/10.1111/ijcp.13966
Gastrointestinal AA amyloidosis secondary to chronic pyelonephritis presenting with refractory diarrhea and severe hypoalbuminemia
  • T Tanaka
  • T Naito
  • Y Midori
  • T Nosaka
  • K Takahashi
  • K Ofuji
  • H Matsuda
  • M Ohtani
  • K Hiramatsu
  • Y Imamura
  • O Yokoyama
  • H Naiki
  • Y Nakamoto
Tanaka, T., Naito, T., Midori, Y., Nosaka, T., Takahashi, K., Ofuji, K., Matsuda, H., Ohtani, M., Hiramatsu, K., Imamura, Y., Yokoyama, O., Naiki, H., & Nakamoto, Y. (2021). Gastrointestinal AA amyloidosis secondary to chronic pyelonephritis presenting with refractory diarrhea and severe hypoalbuminemia. Clinical journal of gastroenterology, 14(6), 1642-1648. https://doi.org/10.1007/s12328-021-01508-1