Clinical characteristics of patients with systemic amyloidosis involved in digestive tract / 中华消化杂志

ABSTRACT

Objective:To study the clinical characteristics and endoscopic manifestations of patients with systemic amyloidosis involved in digestive tract.Methods:Clinical and endoscopic features, biopsy locations and positive rate of patients with systemic amyloidosis involved in digestive tract at Peking Union Medical College Hospital from December, 1991 to December, 2004 (1991 to 2004 diagnosed group, 18 cases) and from March 17th, 2009 to November 4th, 2020 (2009 to 2020 diagnosed group, 47 cases) were retrospectively analyzed and compared. Independent sample t test, Mann-Whitney U test and chi-square test were used for statistical analysis. Results:Among 47 patients in 2009 to 2020 diagnosed group, 41 were primary amyloid light chain (AL) amyloidosis, and the age of onset was older than that of 1991 to 2004 diagnosed group ((57.15±9.10) years old vs. (50.75±10.90) years old), and the time from disease onset to diagnosis was shorter than that of 1991 to 2004 diagnosed group ((13.48±11.72) months vs. (26.38±23.02) months), and the differences were statistically significant ( t=2.08 and -2.13, P=0.048 and 0.047). There was no significant difference in time from disease onset to diagnosis between patients with gastrointestinal manifestations at disease onset and patients without gastrointestinal manifestations at disease onset (10.5 months (6.0 months, 17.3 months) vs.14.0 months (5.8 months, 25.0 months), P>0.05). Among primary AL amyloidosis patients in 2009 to 2020 diagnosed group, weight loss was the most common clinical symptom, the proportion of patients was 87.8%(36/41); secondary was gastrointestinal bleeding, among which 18 patients (43.9%) were stool occult blood test positive and 4 patients (9.8%) were with melena or bloody stool. The endoscopic features of patients with biopsy-confirmed digestive tract involvemen vary, including mucosa hemorrhage and oozing of blood in 4 cases, fragile mucosa and easy to bleed in 3 cases, and 4 cases with bulged mucosa and all the 4 cases of biopsy at bulged mucosa was positive. Gingiva and tongue were the most common biopsy locations and the positive rate of biopsy was ≥60.0%. Rectal mucosa biopsy was performed in 15 patients and 6 were Congo red staining positive. Heart or kidney biopsy was performed in 3 and 6 patients, and the number of positive cases was 2 and 4, respectively. Conclusions:Weight loss and gastrointestinal bleeding are the common gastrointestinal symptoms of primary AL amyloidosis. The common endoscopic manifestations included mucosal bleeding, fragile and bulged mucosa. Biopsy at multiple locations according to experience may help earlier diagnosis and treatment.