Vikram S Gill, Sayi P Boddu, Sami Abujbarah, Kellie L Mathis, Amit Merchea, Justin T Brady
{"title":"炎症性肠病患者的继发性淀粉样变性:三个三级医疗中心的研究结果。","authors":"Vikram S Gill, Sayi P Boddu, Sami Abujbarah, Kellie L Mathis, Amit Merchea, Justin T Brady","doi":"10.1007/s12328-024-02003-z","DOIUrl":null,"url":null,"abstract":"<p><p>Secondary amyloidosis (AA) is a disorder of protein conformation associated with inflammatory disorders. Detailed reports of patients diagnosed with AA and inflammatory bowel disease (IBD) are limited. This study reports the cases of eight patients, across three tertiary medical centers, diagnosed with both IBD and AA between 2000 and 2020. Seven patients had a diagnosis of Crohn disease (CD), while one had ulcerative colitis (UC). All patients were diagnosed with AA after being diagnosed with IBD (median: 15 years later). The small bowel (62.5%) and the colon (62.5%) were the most common IBD locations. 4 patients had undergone TNF-alpha inhibitor therapy and all CD patients required surgical treatment of their IBD. A history of fistula or abscess was identified in 5 patients. The most common initial site of AA was the kidney (75%). All 8 patients presented with some form of renal dysfunction and proteinuria (median: 1500 mg/24 h). Hypoalbuminemia was found in most patients. Six patients developed chronic kidney disease and 4 required dialysis. Anti TNF-alpha antibody therapy led to rapid improvement of renal function in one of four patients who received it. Three patients required a renal transplant. Four patients had died upon the latest follow-up (5-year survival: 75%). The presence of proteinuria, fistula, or abscess should serve as indicators for potentially increased AA risk in CD patients.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Secondary amyloidosis in inflammatory bowel disease patients: findings from three tertiary medical centers.\",\"authors\":\"Vikram S Gill, Sayi P Boddu, Sami Abujbarah, Kellie L Mathis, Amit Merchea, Justin T Brady\",\"doi\":\"10.1007/s12328-024-02003-z\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Secondary amyloidosis (AA) is a disorder of protein conformation associated with inflammatory disorders. Detailed reports of patients diagnosed with AA and inflammatory bowel disease (IBD) are limited. This study reports the cases of eight patients, across three tertiary medical centers, diagnosed with both IBD and AA between 2000 and 2020. Seven patients had a diagnosis of Crohn disease (CD), while one had ulcerative colitis (UC). All patients were diagnosed with AA after being diagnosed with IBD (median: 15 years later). The small bowel (62.5%) and the colon (62.5%) were the most common IBD locations. 4 patients had undergone TNF-alpha inhibitor therapy and all CD patients required surgical treatment of their IBD. A history of fistula or abscess was identified in 5 patients. The most common initial site of AA was the kidney (75%). All 8 patients presented with some form of renal dysfunction and proteinuria (median: 1500 mg/24 h). Hypoalbuminemia was found in most patients. Six patients developed chronic kidney disease and 4 required dialysis. Anti TNF-alpha antibody therapy led to rapid improvement of renal function in one of four patients who received it. Three patients required a renal transplant. Four patients had died upon the latest follow-up (5-year survival: 75%). The presence of proteinuria, fistula, or abscess should serve as indicators for potentially increased AA risk in CD patients.</p>\",\"PeriodicalId\":10364,\"journal\":{\"name\":\"Clinical Journal of Gastroenterology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2024-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Journal of Gastroenterology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1007/s12328-024-02003-z\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/6/16 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Journal of Gastroenterology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s12328-024-02003-z","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/6/16 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
继发性淀粉样变性(AA)是一种与炎症性疾病相关的蛋白质构象紊乱。有关被诊断患有 AA 和炎症性肠病(IBD)的患者的详细报告非常有限。本研究报告了 2000 年至 2020 年间在三家三级医疗中心诊断出同时患有 IBD 和 AA 的八名患者的病例。其中七名患者被诊断为克罗恩病(CD),一名患者被诊断为溃疡性结肠炎(UC)。所有患者都是在确诊 IBD 后才被诊断为 AA(中位数:15 年后)。小肠(62.5%)和结肠(62.5%)是最常见的IBD部位。4名患者接受过TNF-α抑制剂治疗,所有CD患者的IBD都需要手术治疗。5名患者有瘘管或脓肿病史。最常见的AA初始部位是肾脏(75%)。所有 8 名患者都出现了某种形式的肾功能障碍和蛋白尿(中位数:1500 毫克/24 小时)。大多数患者都存在低白蛋白血症。6名患者发展为慢性肾病,其中4人需要透析治疗。在接受抗 TNF-α 抗体治疗的四名患者中,有一人的肾功能得到迅速改善。三名患者需要进行肾移植。最近一次随访时,4名患者已经死亡(5年存活率:75%)。蛋白尿、瘘管或脓肿的出现应作为 CD 患者潜在 AA 风险增加的指标。
Secondary amyloidosis in inflammatory bowel disease patients: findings from three tertiary medical centers.
Secondary amyloidosis (AA) is a disorder of protein conformation associated with inflammatory disorders. Detailed reports of patients diagnosed with AA and inflammatory bowel disease (IBD) are limited. This study reports the cases of eight patients, across three tertiary medical centers, diagnosed with both IBD and AA between 2000 and 2020. Seven patients had a diagnosis of Crohn disease (CD), while one had ulcerative colitis (UC). All patients were diagnosed with AA after being diagnosed with IBD (median: 15 years later). The small bowel (62.5%) and the colon (62.5%) were the most common IBD locations. 4 patients had undergone TNF-alpha inhibitor therapy and all CD patients required surgical treatment of their IBD. A history of fistula or abscess was identified in 5 patients. The most common initial site of AA was the kidney (75%). All 8 patients presented with some form of renal dysfunction and proteinuria (median: 1500 mg/24 h). Hypoalbuminemia was found in most patients. Six patients developed chronic kidney disease and 4 required dialysis. Anti TNF-alpha antibody therapy led to rapid improvement of renal function in one of four patients who received it. Three patients required a renal transplant. Four patients had died upon the latest follow-up (5-year survival: 75%). The presence of proteinuria, fistula, or abscess should serve as indicators for potentially increased AA risk in CD patients.
期刊介绍:
The journal publishes Case Reports and Clinical Reviews on all aspects of the digestive tract, liver, biliary tract, and pancreas. Critical Case Reports that show originality or have educational implications for diagnosis and treatment are especially encouraged for submission. Personal reviews of clinical gastroenterology are also welcomed. The journal aims for quick publication of such critical Case Reports and Clinical Reviews.