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Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience
A Young Lim1, Ji Hyeon Lee1, Ki Sun Jung1
1Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
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.
Insights
Gastrointestinal amyloidosis, particularly AL amyloidosis, is linked to poorer prognoses and more organ involvement. While GI involvement impacts survival, it wasn't a significant predictor in multivariate analysis for AL amyloidosis.
Area of Science:
- Gastroenterology
- Hematology
- Pathology
Background:
- Systemic amyloidosis frequently affects the gastrointestinal (GI) tract.
- Data on GI amyloidosis clinical features and outcomes are limited.
- This study investigates pathologically proven GI amyloidosis cases.
Purpose of the Study:
- To describe the clinical characteristics of patients with systemic amyloidosis and GI involvement.
- To evaluate the outcomes and survival rates of patients with GI amyloidosis.
- To identify prognostic factors in GI amyloidosis, particularly AL amyloidosis.
Main Methods:
- Retrospective analysis of 155 systemic amyloidosis patients diagnosed between April 1995 and April 2013.
- Identification of 24 patients (15.5%) with biopsy-confirmed GI amyloidosis.
- Comparison of clinical features and survival between patients with and without GI involvement.
Main Results:
- Amyloidosis light chain (AL) amyloidosis was the most common type (83.3%) in GI cases.
- Diarrhea (45.8%), anorexia (37.5%), and weight loss were the most frequent GI symptoms.
- Patients with GI involvement showed greater organ involvement and a trend towards shorter overall survival (OS).
Conclusions:
- GI involvement in AL amyloidosis is associated with poorer prognosis and more advanced disease.
- Patients with GI amyloidosis often present with multi-organ involvement.
- Further research is needed to clarify the prognostic significance of GI involvement in systemic amyloidosis.
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