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Systemic amyloidosis and the gastrointestinal tract
Prayman T Sattianayagam1, Philip N Hawkins, Julian D Gillmore
1National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine (Royal Free Campus), University College London Medical School, London, UK.
Nature Reviews. Gastroenterology & Hepatology
|September 3, 2009
Summary
Systemic amyloidosis involves abnormal protein buildup in the body, often affecting the gastrointestinal tract with nonspecific symptoms. Current treatments focus on reducing precursor proteins, with limited options for gastrointestinal issues.
Area of Science:
- Gastroenterology
- Internal Medicine
- Pathology
Background:
- Systemic amyloidosis is defined by extracellular fibrillar protein deposits, with diverse precursor proteins causing different types.
- Gastrointestinal tract involvement is common in systemic amyloidosis, frequently subclinical, but can also be a primary cause, like AA amyloidosis secondary to Inflammatory Bowel Disease (IBD).
Purpose of the Study:
- To review the clinical presentations, diagnostic features, and management strategies for gastrointestinal manifestations of systemic amyloidosis.
- To highlight the nonspecific nature of gastrointestinal symptoms and endoscopic findings in systemic amyloidosis.
- To discuss current therapeutic aims and available treatments for gastrointestinal symptoms.
Main Methods:
- Literature review of systemic amyloidosis with a focus on gastrointestinal involvement.
- Analysis of clinical presentations, diagnostic modalities, and treatment outcomes from existing case reports and series.
- Synthesis of information regarding the pathophysiology and management of gastrointestinal symptoms.
Main Results:
- Gastrointestinal symptoms are varied and nonspecific, including macroglossia, hemorrhage, motility disorders, altered bowel habits, and malabsorption.
- Endoscopic and radiological findings are also nonspecific, with the small intestine being the most frequently affected site.
- No specific treatments for gastrointestinal symptoms exist; supportive care like nutritional support for motility disorders and pharmacotherapy for diarrhea are suggested.
Conclusions:
- Systemic amyloidosis frequently impacts the gastrointestinal tract, presenting with nonspecific symptoms and findings.
- Therapeutic strategies primarily aim to reduce the precursor protein, with limited specific interventions for gastrointestinal manifestations.
- Management of gastrointestinal symptoms relies on supportive measures, and surgery is reserved for emergencies due to high risks.
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