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Gastrointestinal Symptoms

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Amyloidosis and Fabry Disease

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Abstract

Amyloidosis is a rare disease with the potential to affect multiple organ systems, including the gastrointestinal (GI) tract. The course of the disease includes highly variable systemic clinical signs and symptoms, which could initiate with numerous nonspecific GI symptoms and increase the risk of misdiagnosis. Clinical manifestations include dysmotility, malabsorption, diarrhea, constipation, bowel obstruction, bleeding, and rarely jaundice or portal hypertension. Radiologic findings are typically nonspecific. The two pillars of treatment for amyloidosis consist of drugs to alleviate GI symptoms and drugs to reduce systemic disease progression. The patient’s nutritional status should always be a concern throughout the follow-up. Surgery is not recommended in bowel pseudo-obstruction, as it can cause further complications without any significant benefit. In the rare cases of only localized GI amyloidosis without systemic involvement, observation without initiating treatment has been the preferred modality.

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Authors and Affiliations

  1. Department of Gastroenterology and Hepatology, Federal University of the State of Rio de Janeiro—UNIRIO, Rio de Janeiro, RJ, Brazil

    Cibele Franz, Amanda Saavedra, Agueda Miranda & Márcia Lyrio

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  1. Cibele Franz
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  2. Amanda Saavedra
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  3. Agueda Miranda
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Editors and Affiliations

  1. Postgraduate Program in Cardiovascular Science, Fluminense Federal University, Niteroi, Rio de Janeiro, Brazil

    Diane Xavier de Ávila

  2. Cardiology Division, Fluminense Federal University, Niterói, Rio de Janeiro, Brazil

    Humberto Villacorta Junior

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Franz, C., Saavedra, A., Miranda, A., Lyrio, M. (2023). Gastrointestinal Symptoms. In: Xavier de Ávila, D., Villacorta Junior, H. (eds) Amyloidosis and Fabry Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-17759-0_11

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  • DOI: https://doi.org/10.1007/978-3-031-17759-0_11

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