Korean J Intern Med.  2015 Jul;30(4):496-505. 10.3904/kjim.2015.30.4.496.

Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience

• Lim AY
• Lee JH
• Jung KS
• Gwag HB
• Kim DH
• Kim SJ
• Lee GY
• Kim JS
• Kim HJ
• Lee SY
• Lee JE
• Jeon ES
• Kim K

• KMID: 2366010
• DOI: http://doi.org/10.3904/kjim.2015.30.4.496

Abstract

BACKGROUND/AIMS
The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis.
METHODS
We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy.
RESULTS
Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447).
CONCLUSIONS
The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.

Keyword

Gastrointestinal amyloidosis; Amyloidosis; Gastrointestinal diseases

MeSH Terms

Adult
Aged
Amyloid Neuropathies, Familial/*diagnosis/immunology/mortality/pathology/therapy
Biomarkers/analysis
Biopsy
Female
Gastrointestinal Diseases/*diagnosis/immunology/mortality/pathology/therapy
Gastrointestinal Tract/immunology/*pathology
Humans
Immunoglobulin Heavy Chains/analysis
Immunoglobulin Light Chains/analysis
Kaplan-Meier Estimate
Male
Middle Aged
Multivariate Analysis
Predictive Value of Tests
Prognosis
Proportional Hazards Models
Republic of Korea
Retrospective Studies
Risk Factors
Serum Amyloid A Protein/analysis
Time Factors
Biomarkers
Immunoglobulin Heavy Chains
Immunoglobulin Light Chains
Serum Amyloid A Protein